Lipoid adrenal hyperplasia presents with DSD in men since, in the absence of StAR, Leydig cells are affected and cannot produce adequate concentrations of androgens, presenting testicular insufficiency that leads to hypogonadism with alteration in the virilization of the Wolffian ducts, the urogenital sinus and the external genitalia. The gene discussed is STAR; the disease is disorder of sexual differentiation.