Diagnosis and treatment of a patient with a congenital anomaly of the midgut with persistent symptom in adult can be difficult because it is particularly in adults. Reversed rotation of the midgut is the rarest of all malrotation anomalies. We report a case of reversed intestinal rotation and review the embryology, clinical presentation, and radiographic findings of this disorder. Although this anomaly is rare, it may be diagnosed by the knowledge of embryology and anatomy.