Developmental defect of Mullerian duct is a congenital malformation characterized by anomalies in famale upper genital organs. As the anomaly is associated with obstruction of the duct, other congenital anomalies of urinary tract specially ipsilateral ranal agenesis are common.

This anomaly is often discovered in adolescent period and is usually asymptomatic until puberty. To discover this anomaly as soon as possible, whenever renal agenesis is associated with pelvic mass, Mullerian duct obstructive anomaly should be in mind and ruled out.

Our patient was a 15-years-old female with cheif complaint of abdominal and pelvic pain off and on. After Complete work up a diagnostic laparatomy was performed which revealed a double uterus associated with an obstructed hemivagina and ipsilateral renal agenesis.

Combination of these anomalies were compatible with diagnosis of unilateral Mullerian duct defect.