Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare disease of unknown etiology characterized by ischemia of intestinal segments, occuring due to intimal proliferation of the mesenteric veins and partial blockage of blood drainage. Diagnosis is performed pathologically and definitive treatment is surgical, where involved segments of the intestine are resected. Here we describe a case in which the patient underwent a comprehensive medical evaluation, finally diagnosed with IMHMV after bowel resection. The purpose of this case report is to present the diagnostic challenge to clinicians and raise awareness to this condition.