Androgen biosynthetic defects high in the steroid pathway such as lipoid congenital adrenal hyperplasia (CAH); caused by an abnormality in the steroidogenic acute regulator (StAR) protein) can result in a similar biochemical picture to that of gonadal dysgenesis with low basal and stimulated testosterone levels and low testosterone precursors. Here, STAR is linked to congenital lipoid adrenal hyperplasia due to STAR deficency.