The combination of disorder of sex development (DSD) and canal of Nuck hernia, in which the hernial sac contains the internal reproductive organs (gonads) of both genders, is exceedingly rare. We present a unique case of a neonate who presented with ambiguous genitalia and a lump in the left inguinal region. The child underwent various radiographic procedures and hernial repair. Blood workup, karyotyping, and histopathologic analysis from gonads confirmed the diagnosis of Ovotesticular disorder of sex development. Subsequently, the child had a reconstructive vaginoplasty, and the final decision regarding gender assignment will be made after assessing mental and sexual behavior in early childhood.