OBJECTIVES: To report the case of a 23-yearoldpatient with ambiguous external genitalia, gynecomastiaand cyclic hematuria who demanded proper investigationsand actions to be considered male.METHODS: A set of tests were performed: history and physicalexamination, blood tests, hormonal determinations, nuclearchromatin, cariotype, renal and pelvic ultrasound, voidingcystourethrogram (VCUG), laparoscopy, exploratory laparotomy,left hystero-salpingo-oophorectomy, right orchydopexy, testicularbiopsy and fibrous cord section.RESULTS: History and physical examination showed a longslim individual with male fat distribution, female pubic hairdistribution, Tanner`s grade 4 gynecomastia with scarce breasthair, absence of scrotal gonads, and urogenital sinus. Bloodtests were normal. Hormonal tests were normal. There wereno anomalies in the renal ultrasound and a small uterus wasfound in the pelvic ultrasound. The chromatin test was positiveand cariotype showed a 46/XX cell line.The VCUG only contrasted a normal posterior urethra.Laparotomy discovered a small uterus with two fallopiantubes: the left one was dilated with a polycystic ovary, and theright one was hypoplastic with testicle, vas deferens, andepididymis near it. Right orchydopexy was carried out afterbiopsy and section of the fibrous cord. Androgen therapy,subcutaneous mastectomy, and urethroplasty were performedpostoperatively with satisfactory results.CONCLUSIONS: Severe cases of genital anomalies may besolved after a good psychological evaluation and propersurgical repair.