Cryptorchidism and persistence of Müllerian remnants in a normal male

A four-year-old boy was explored for bilateral cryptorchidism. Operation led to the discovery of two intraabdominal testes with their corresponding vasa, two Fallopian tubes and a small uterus. No urological anomalies were found except for a vesico-ureteral reflux. Cystourethroscopy revealed a normal male tract. Sexual chromatine was negative and karyotype was 46, XY. A normal rise in testosterone serum level was recorded after stimulation with CGH. At operation both gonads could be placed extra-abdominally and the female internal structures were biopsied and left in place. This very unusual condition is derived from the absence of regression of Müllerian structures during fetal life due to a deficit in testicular secretion of anti-Müllerian hormone. The current knowledge on this topic is updated.