This is one of the few cases of sexual ambiguity included in this book depicting an apparent healthy young female, evaluated for primary amenorrhea, who was found to have bilateral inguinal lumps and absent uterus/ovaries on pelvic scan. Thorough biochemical investigations, followed by detailed anatomic imaging, suggested a case of 46 XY DSD resulting from Leydig cell hypoplasia (LCH)/testosterone biosynthetic defect. The final diagnosis was established by a histopathology report of the resected inguinal testes typical of LCH, though her genetic mutational analysis was inconclusive. Thereafter, she underwent bilateral orchidectomy and subsequent estrogenization.