Abstract
Introduction: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is an inherited autosomal recessive condition (AR). MMIHS is a rare disease that is characterized by functional urinary bladder obstruction and functional intestinal obstruction. MMIHS is a fatal congenital disease with a poor prognosis. Case presentation: We report the cases of two MMIHS patients who were referred to us due to esophageal perforation in the first patient and prenatal distended urinary bladder and stomach and abdominal wall weakness in the second patient. Conclusion: MMIHS is a rare inheritance syndrome that is associated with morbidity and mortality, and patients usually die within the first year of life.