ABSTRACT
Gonadoblastoma typically occurs in dysgenetic gonads, but may rarely also involve a normal ovary or testis. Approximately 40% of such tumors are bilateral. Among affected individuals, up to 80% are phenotypic females and the rest are phenotypic males. Most patients with gonadoblastoma have 46,XY karyotype or various forms of mosaicism. The gonads are usually abnormal, with hypospadias, cryptorchidism and internal female secondary sex organs, which are either in the inguinal region or intra- abdominally. Gonadoblastomas are considered to be clinically benign neoplasms, but up to 50% are accompanied by foci of malignant germ cell tumor, mostly seminoma, and occasionally yolk sac tumor, embryonal carcinoma, choriocarcinoma or teratoma. This paper presents a brief literature review based on a case of an XY female reared as male, with development of a gonadoblastoma in the left ovary.

IMPACT STATEMENT
Gonadoblastomas are rare gonadal tumours, which are usually benign but they may sometimes become malignant if not treated. This paper presents a case report of gonadoblastoma in a case of intersex followed by a brief literature review.