Primary pigmented nodular adrenocortical disease is a rare cause of Cushing's syndrome in children and young adults. It is characterized by hypercorticolism resistant to dexamethasone suppression and at microscopic examination by multiple small black cortical nodules containing large cells with eosinophilic cytoplasm and lipofuscin with internodular cortical atrophy. Its pathogenesis is unknown. Bilateral adrenalectomy is the treatment of choice. We report a case of Cushing's syndrome due to primary pigmented nodular adrenocortical disease in a 32 year old female and review the literature.