Carney complex is a multiple endocrine neoplasia syndrome with various features which include myxomas, endocrine tumours and lentigines lesions. We report a case of Carney complex with components of lentigines, ACTH independent adrenal Cushing’s syndrome (with a paradoxical increase in 24 hour urinary cortisol following the high dose (8mg) dexamethasone suppression test – and is likely to be due to primary pigmented nodular adrenal hyperplasia) positive for a protein kinase A type 1A regulatory subunit (PRKAR1A) gene mutation.