Taken together with the emerging experience from non-classic congenital lipoid adrenal hyperplasia due to STAR defects, these reports highlight a relation between enzyme function and phenotype and suggest that human adrenal function is more sensitive to partial disruption of STAR and CYP11A1 than gonadal (testis) function. Here, STAR is linked to congenital lipoid adrenal hyperplasia due to STAR deficency.