A 63-year-old Chinese woman presented to our facility with a history of bilateral photopsia and blurred vision for the last two months. She had no history of seizure, vomiting, head injury, or exposure to medications that could cause such effects. A thorough ophthalmological and systemic examination was carried out. An ocular examination showed best corrected visual acuity of 20/100 in the right eye and 2/80 in the left eye. A fundoscopic examination revealed the presence of multiple choroidal masses in both eyes. An ultrasonographic evaluation of the eye demonstrated elevated choroidal masses in both eyes with a maximal elevation of 3.2mm. A physical examination showed skin nodules over the chest and abdominal areas. The pathology of the skin nodules revealed metastatic clear cell adenocarcinoma of unknown origin. Computerized tomography of the chest, abdomen and pelvic area was performed. A mass in the left upper lobe of the lung with several enlarged mediastinal lymph nodes was detected. Moreover, skin nodules were found on the chest and abdominal walls, and a left adrenal nodule was also present. A bone scan revealed multiple metastases. Our patient then underwent a video-assisted thoracic surgical (VATS) biopsy to obtain adequate material to establish a pathological diagnosis. Histologically, the tumors from lung parenchyma, pleura and mediastinal lymph nodes were infiltrated by core-like or abortive glandular structures that consisted of pleomorphic clear tumor cells with foamy cytoplasm and distinct nucleoli. Immunohistochemical (IHC) staining test results showed that the tumor cells were positive for pancytokeratin (AE1/AE3), cytokeratin 7 (CK-7), thyroid transcription factor 1 (TTF-1) and carcinoembryonic antigen (CEA). The results for Ki-67 staining displayed a proliferative index of approximately 45 to 50 percent. Results of a histochemical stain showed tumor cells positively stained by periodic acid Schiff (PAS) and PAS with diastase indicated the presence of glycogen In contrast, the tumor cells tested negative for CK-5/6, CK-20, vimentin, thyroglobulin, CD10, CDX2, epithelial membrane antigen (EMA), transcription factor E3 (TFE-3), α-inhibin, Hep-par-1, glypican-3, p63 and HMB-45 (data not shown). The clinical and pathological features of our patient’s case were compatible with a clear cell adenocarcinoma of the lung (T2bN2M1b, stage IV, according to the American Joint Committee on Cancer (AJCC) cancer staging guide, seventh edition). Systemic chemotherapy with pemetrexed (500mg/m2, every three weeks) and cisplatin (75mg/m2, every three weeks) was initiated. After cycle six of chemotherapy, a fundoscopic examination showed near complete resolution of the choroidal masses, coinciding with improvement in her vision. Although her cancer status outside the orbit remained stable, she had a good quality of life with no chemotherapy maintenance for over 24 months at the time of this report.