Pregnancy in a true hermaphrodite

True hermaphroditism is a rare form of intersexuality, and fertility in such an anomaly has been reported only once before in literature. A 19-year-old true hermaphrodite reared as a female with normal secondary sex characteristics and a karyotype of 46,XX was treated by removal of the left ovotestis. The right ovary and other pelvic organs were of a normal female. Ten months after the treatment she became pregnant. The pregnancy was complicated by a premature delivery of a 1500-g male infant that showed no congenital anomalies. Histologic findings of the ovotestis and clinical findings of a true hermaphrodite with pregnancy are presented.