Hernia uteri inguinalis in ovotesticular disorder of sexual differentiation: A rare complication and role of imaging

Neonate with ambiguous genitalia can cause great apprehension for the family as well as for healthcare providers. We report a rare complication of delayed diagnosis of hernia uteri inguinalis in ovotesticular disorder of sexual differentiation (DSD) in 20-year-old male patient who presented with pain and swelling in left inguinal region since 1 month. He had a past surgical history of repair of hypospadias 10 years back. On imaging, the left inguinal hernia sac contained nonfunctioning uterus and one ovary in the left scrotal sac and one testis in the right scrotal sac. Further investigation confirmed genotypically female (46XX) with negative sex determining region-Y gene on fluorescence in situ hybridization. The patient was given psychiatric counseling and wished to remain as male. The left inguinal hernia was repaired with excision of nonfunctioning uterus, ovary, and fallopian tube. Hernia uteri inguinalis is rare complication seen in DSD with only three cases being reported worldwide thus far, including our case.