An Unusual Clinical and Radiological Presentation of Ovotesticular Disorders of Sex Development with Male and Female External Genitalia: A Case Report

Background: Ovotesticular disorders of sex development (ODSD), previously known as true hermaphroditism, is a rare disorder sexual differentiation that causes ambiguity in external genitalia and the presence both ovarian testicular elements gonads. The condition characterised by existence tissue with distinctive tubules same gonad (ovotestis), or separately an individual. most common cytogenetic karyotype 46,XX. age presentation symptoms vary, but typically include hypospadias, urogenital sinus, inguinal hernia, cryptorchidism, gynaecomastia during peripubertal age, well lower abdominal mass. Case Presentation: A 15-year-old female exhibited typical male genitalia, including normal penile shaft, scrotal sac, palpable testes, urethral opening, separate vaginal opening located between shaft scrotum. Pelvic MRI confirmed testes within sac infantile-sized uterus central endometrium, left-sided ovary pelvic region. right was not seen. Correlative ultrasound these findings indicated had echo texture vascularity. patient diagnosed ODSD, further hormonal testing recommended. Conclusions: ODSD cause genital ambiguity. Typically, paramesonephric mesonephric duct derivatives are seen, affected individuals present neonates infants ambiguous genitalia. At unilateral bilateral gynaecomastia. Fertility uncommon has been reported female-reared patients. ovotestis commonest people to be predisposed germ cell tumours. Over 500 cases have literature. Ultrasound first line investigation used see organs. non-invasive test choice for detecting pelvis. purpose this paper describe various clinical radiological features associated ODSD.