Disorders of Sex Development (DSD), formerly known as sexual ambiguity, are abnormalities leading to discrepancy between internal genital organs (IGO), external genitals (EGO) and secondary sexual characteristics. We report on a particularly complex case of 46 XY gonadal dysgenesis, monitored at the childrens hospital of Rabat, which presented a male phenotype and whose parents consult for bilateral cryptorchidism, with well-individualized male external genital organs, a male morphotype without any other clinically detectable malformation. Our goal is to contribute to the study of the rare peculiarities of sexual ambiguities, to offer a diagnostic approach to their management.