Müllerian duct agenesis and other congenital anomalies

The Rokitansky anomalad, although a rare disorder, ranks second as a cause of primary amenorrhea in adolescents. This patient presented with a congenital absence of the vagina and uterus and other associated minor abnormalities. She had primary amenorrhea and asynchronic pubertal development. The syndrome appears to be related to a defect in the paramesonephric ducts with frequent involvement of adjacent structures. This report reviews the syndrome and emphasizes the importance of the chronological sequence of events during female sexual development.