The dose was gradually decreased to 15 mg/m<sup>2</sup>/day PO BID, with clinical improvement and normalization of the serum ACTH level.<h4>Conclusions</h4>The autosomal recessive glucocorticoid deficiency, a variant of FGD type 4, is a very rare condition that may lead to high rates of mortality when the diagnosis and treatment occur late. Here, POMC is linked to familial glucocorticoid deficiency.