BACKGROUND: Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare disease characterized by narrowing of the lumen caused by mesenteric vein intimal hyperplasia, resulting in chronic intestinal ischemia. Although the colorectum is the primary site affected by this condition, involvement of the small intestine is even more rare. Recurrence of IMHMV after surgical resection of the affected bowel is uncommon. CASE SUMMARY: In this case report, we present a unique instance of IMHMV exclusively occurring in the small intestine. The patient experienced small intestinal perforation, infarction, and obstruction before receiving a clear diagnosis, which was achieved only during the third operation. In this review, we analyzed 84 reported cases to summarize the etiology, clinical manifestations, and diagnostic challenges of IMHMV, with the aim of raising awareness regarding this rare condition among clinicians. CONCLUSION: Notably, IMHMV can also affect the small intestine alone. When refractory enteritis with endoscopic findings of nonspecific ischemic changes is encountered, IMHMV should be considered for potential diagnosis.