Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare cause of segmental intestinal ischemia. It is usually associated with chronic symptoms of abdominal pain, diarrhea, and rectal bleeding, but it also can present in more acute and dramatic clinical presentations. Surgical segmental bowel resection is the proper treatment, but it is commonly delayed by the misdiagnosis of this condition as inflammatory bowel disease, such as Crohn's or idiopathic ulcerative colitis. Awareness of IMHMV as a non-thrombotic cause of intestinal ischemia may prevent unnecessary medical treatment with topical and systemic steroids, antibiotics, and even mesalazine and avoid potentially fatal complications of the disease. We report a case with early development of severe complications and also review the literature on IMHMV.