We documented a new case of 46,XY true hermaphroditism in an 18-year-old patient, reared as a female at birth, with ambiguous genitalia and primary amenorrhea. At laparotomy, bilateral dysgenetic ovotestis containing gonadoblastoma were found. Karyotypes of peripheral lymphocytes and cells from tissue of both gonads were all 46,XY. Plastic surgery was conducted to transform ambiguous genitalia to the full female phenotype. Clitoreduction by glans resection and fastening of the corpora cavernosa in the labia major with enlargement of the vaginal orifice were performed. The results was a small eretcile organ with preserved sensitivity and a female capable of normal sexual activity.