An empty sella is defined as a sella which, regardless of its size, is completely or partly filled with cerebrospinal fluid. The endocrine function of primary empty sella syndrome is usually normal, but sometimes this syndrome is associated with complete or partial pituitary insufficiency and rarely hypersecretion of pituitary hormone. Primary empty sella syndrome combined with Cushing's disease has rarely been reported. A 45-years-old woman presented with cushingoid feature. Her urinary cortisol and 17-hydroxycorticosteroid excretion were increased. The results of endocrine function testing were suggestive of Cushing's disease. Sella MRI showed of partially empty sella and pituitary microadenoma. The pituitary microadenoma was removed by the trans-sphenoidal approach. We report here on this case together with a review of the literature.