[True hermaphroditism]

To report the case of a 23-year-old patient with ambiguous external genitalia, gynecomastia and cyclic hematuria who demanded proper investigations and actions to be considered male.

A set of tests were performed: history and physical examination, blood tests, hormonal determinations, nuclear chromatin, cariotype, renal and pelvic ultrasound, voiding cystourethrogram (VCUG), laparoscopy, exploratory laparotomy, left hystero-salpingo-oophorectomy, right orchydopexy, testicular biopsy and fibrous cord section.

History and physical examination showed a long slim individual with male fat distribution, female pubic hair distribution, Tanner's grade 4 gynecomastia with scarce breast hair, absence of scrotal gonads, and urogenital sinus. Blood tests were normal. Hormonal tests were normal. There were no anomalies in the renal ultrasound and a small uterus was found in the pelvic ultrasound. The chromatin test was positive and cariotype showed a 46/XX cell line. The VCUG only contrasted a normal posterior urethra. Laparotomy discovered a small uterus with two fallopian tubes: the left one was dilated with a polycystic ovary, and the right one was hypoplastic with testicle, vas deferens, and epididymis near it. Right orchydopexy was carried out after biopsy and section of the fibrous cord. Androgen therapy, subcutaneous mastectomy, and urethroplasty were performed postoperatively with satisfactory results.

Severe cases of genital anomalies may be solved after a good psychological evaluation and proper surgical repair.