True hermaphrodite with teratoma

A 32 years old healthy male, married for 6 years presented with infertility. Clinical examination revealed normal external genitalia with underdeveloped empty scrotum. Radiological investigations reported bilateral intra abdominal testis with left testis showing features of teratoma. Intra-operative findings showed bilateral intra abdominal testis along with uterus and fallopian tube and left testis with features of teratoma. Histopathology confirmed the presence of uterus, cervix, right adnexa with atropic testis with tube, seminal vesical, epididymis, and left adnexa ovotestis with mature teratoma, tube and epididymis. Cytogenetics reported 46, XY karyotype. This case is rare presentation of combination of male phenotype true hermaphrodite with presence of teratoma.