A 51-year old male was investigated for chronic diarrhea, episodes of moderate diffuse abdominal pain and 10-kg weight loss. On physical examination, the patient presented muscle wasting, without any fever, hepatosplenomegaly or jaundice. The stool studies were positive for steatorrhea. The laboratory workup revealed moderate iron deficiency anemia, signs of hyposplenism: Howell-Jolly bodies on the peripheral blood smear, elevated platelet count, hypocalcaemia and an elevated alkaline phosphatase level. The serum endomysial and tissue transglutaminase IgA antibodies were positive in high titre. Total villous atrophy, crypt hyperplasia, increased intraepithelial lymphocytes and increased plasma cells and lymphocytes in the lamina propria were found on the duodenal biopsy performed by upper digestive endoscopy. The intraepithelial lymphocytes were small, without atypical features. The immunohistochemistry testing found intraepithelial lymphocytes positive for CD3 and few lymphocytes positive for CD8 in lamina propria; CD30 staining revealed isolated positive cells in lamina propria. The abdominal ultrasound revealed fluid-distended small bowel loops with an enlarged, hyper echoic mesentery, anechoic cysts corresponding to mesenteric lymph nodes and a reduced spleen size. We established a diagnosis of celiac disease complicated with CMLNS. A gluten-free diet was recommended and a three-month monitoring schedule proposed. Three months later, the patient complained of persistent symptoms. The stool examination revealed a Yersinia enterocolitica infection. The patient received adequate antibiotic therapy, resulting in stool sterilization. After six months of gluten-free diet, the clinical manifestations were similar, despite diet adherence, confirmed by the decline in tissue transglutaminase IgA titre. An intestinal lymphoma was suspected and capsule endoscopy performed, which investigated the entire small bowel. This examination revealed an atrophic villous pattern in the proximal jejunum, without mucosal changes suggestive of lymphoma; a “bulging” mass with normal mucosal surface was described and was interpreted as compression from a mesenteric lymph node. The entire small bowel was investigated by capsule endoscopy. Mesenteric cystic masses with central low attenuation and a thin enhancing rim were found on oral and IV-contrast enhanced computed tomography (CT). No small bowel wall segmental enlargements were present on CT enteroclysis. As the clinical suspicion of malignancy was still high, a contrast-enhanced ultrasound (CEUS) was considered in order to describe the vascular pattern of the mesenteric tissue. After peripheral venous injection of 4.8 ml of ultrasound contrast agent (Sonovue), an arterial rim enhancement was seen around necrotic lymph nodes, without washout of the contrast agent in the venous phase. Some of the investigated masses had septa exhibiting the same vascular pattern, suggesting an anarchic vascularization. A diagnostic laparoscopy was performed with removal of two lymph nodes. These cystic masses were found to contain a milky fluid. The histopathological examination of the samples revealed central homogeneous acidophilic material, fibrotic walls with a rim of normal lymphocytes at the periphery of necrotic lymph nodes and no signs of malignancy or infection. The gluten-free diet and monitoring was continued. After another five months, the patient presented with fever (39°Celsius) and severe liver failure. The blood cultures were negative. The serological markers for viral or autoimmune hepatitis and leptospirosis were also negative. The already severe clinical status worsened, with the development of hepatic encephalopathy and severe upper gastrointestinal bleeding. Despite intensive supportive treatment, the patient died 48 hours after admission. On necropsy, many nodular grayish-white masses, either fluctuant or firm, containing a milky fluid, were found in the mesentery. The microscopic examination of necrotic mesenteric lymph nodes revealed a central homogeneous acidophilic material, fibrotic walls and rare lymphocytes and plasmocytes in the periphery. Infiltration of abnormal T-cell lymphocytes, with atypical nuclear features, was present in the surrounding adipose tissue. The immunohistochemistry testing was positive for CD3 and CD30. The same infiltrative tumor cells were present in the liver, spleen, gastric walls, kidney, lungs and bone marrow; no malignant cells were present in the small bowel samples examined.