A 49-year-old female patient presented to our hospital with a solitary lesion in her liver, which was found accidentally by an abdominal ultrasonic scan during a physical examination. This patient was asymptomatic, and physical examination of this patient showed no positive signs. The patient had no history of viral hepatitis and alcohol abuse. She had a 6-year history of diabetes and her blood glucose level was not controlled well. The blood pressure of the patient was normal. The patient found this lesion with its diameter of 2 cm in her checkup 2 years before, and she refused further examination and chose follow-up at that time. The patient had undergone breast operation 20 years before and the pathological result of the resected breast lesion was benign. The results of laboratory tests were almost in the normal range except blood glucose and α-fetoprotein (AFP). Blood glucose was significantly high (13.18 mmol/L) and AFP was a slight elevation (7.32 ng/mL, normal range: 0.605–7.0 ng/mL). The Child-Pugh score was <5. An ultrasonic scan of her liver showed the mass of the lesion to be located in the right lobe of her liver, with a size of 5.7 × 4.9 cm. The shape of the lesion was anomalistic, but its edges could be easily discerned. Magnetic resonance (MR) images showed a mass was located in the segment 7 and 8 and caudate process of the liver. T1-weighted MR images revealed that the mass is low signal intensity, and T2-weighted MR images showed the signal intensity of the mass is high. MR with diffusion-weighted images (MR-DWI) showed that the mass was hyperintensity with restricted diffusion, and MR with perfusion-weighted imaging (MR-PWI) showed the mass was hyper-enhanced in the arterial phase and de-enhanced in the portal phase. Coronal view of 2-dimensional fast Imaging Employing STeady-state Acquisition (2D-FIESTA) sequence images showed the mass is close to the right hepatic vein and the right posterior branch of the portal vein, and MR cholangiopancreatography showed no obvious abnormality in the bile duct. CT images showed the liver mass was hypoattenuating, and was hyperenhanced in the hepatic arterial phase and de-enhanced on delayed phase. The mass was lobulated, but its edges were also discernible. The presence of calcification and expanded bile ducts were not found. On further inspection, the inferior vena cava was observed to exert a pressing force on the mass. We reconstructed 3D images from CT images to show the relationship between the mass and the portal vein, hepatic vein, and their branches. These characteristic results suggested that the mass in the right lobe of the liver was most likely HCC. With sufficient preoperative preparation, the patient underwent surgical resection to remove the mass. During exploration of the abdomen, we discovered the mass to be located at segment 7 and 8, with a diameter of about 6.0 cm. The mass was observed to cling onto the second porta of the liver. Other abnormalities in the abdomen were not observed. Next, we separated the right lobe of the liver and discovered that the blood pressure of the patient suddenly increased to 220/112 mmHg. We immediately paused the operation and the blood pressure decreased to 108/60 mmHg. We noticed that the levels of blood pressure were increased to very high when the tumor was stimulated. We then exposed the retroperitoneal area of the patient and ensured no positive findings in her adrenals were present. Accordingly, we speculated that the tumor had the same characteristic of adrenal PCCs. We used large doses of sodium nitroprusside to control the patient's blood pressure and resected the tumor quickly. The blood pressure suddenly decreased to 35/20 mmHg when the tumor was totally removed, and large doses of noradrenaline were then administrated. Finally, the patient's blood pressure increased to 90/60 mmHg without pressor agents. Histopathological examination of the resected tumor discovered that it had a gray appearance with a diameter of about 6 cm. Hematoxylin-eosin (H&E) staining of the tumor tissues showed that tumor cells were irregular and with pink cytoplasm and abundant granular, sustentacular cells present, with the vascular net surrounding tumor cell nests. Immunohistochemical staining showed that the tumor tissue was positive for neural-related markers such as CD56 (neural cell adhesion molecule), synaptophysin (Syn), chromograninA (CgA), neuron-specific enolase (NSE), S-100, and tissue were also positive for several epithelial or mesenchymal markers including pan-cytokeratin, smooth muscle actin (SMA), and vimentin, whereas the tissues were negative for markers of hepatocytes such as Hepatocyte, Glypican-3, and Arginase-1, and were negative for HMB-45, a marker of melanocytic tumors. The tumor tissue was also negative for some epithelial markers such as ethidium monoacide (EMA), and cytokeratin 19. CD34, and CD31 were stained positive in vessels of tumor tissues, and Ki-67 index was in the low value (<1%). Collectively, these results indicated that the pathological diagnosis of the tumor was PGL. Eight days following the operation, the patient recovered without complications. In addition, blood glucose levels of this patient were decreased to the normal range within 20 days afterwards. Follow-up examination of liver function, blood glucose levels, and an MRI scan of the entire abdomen and 131I-MIBG scan of the patient's body showed no abnormalities. To date, the patient has followed-up for >2 years and no evidence of recurrence or metastasis has been noted.