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Only 50 cases of idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) have been reported since 1991 when it was first described. This rare etiology for chronic colonic ischemia is often debilitating to the patient's quality of life, and no effective medical treatment is available. IMHMV is frequently confused with inflammatory bowel disease because the most common presenting symptoms include abdominal pain, diarrhea, and hematochezia. Surgical resection is curative; however, the diagnosis is rarely reached preoperatively. In the present report, we have described the seventh patient with a diagnosis of IMHMV before surgery and included a literature review to help clinicians recognize this condition., (© 2022 Published by Elsevier Inc. on behalf of Society for Vascular Surgery.)