Results: A 54-year-old male was hospitalized for severe high blood pressure, depression and weight loss. Biological investigation revealed serious hypokaliema, severe ACTH dependent hypercortisolism with elevated urinary free cortisol secretion, loss of diurnal variation, and excess plasma ACTH level. CT-scan revealed a nodular lesion in the right adrenal gland with hyperplasia in the left one. The nodular lesion was assumed to be a pheochromocytoma based on the elevated serum and urinary catecholamine and metabolites and local uptake in right adrenal gland in 131 I-MIBG scan. Right adrenalectomy was performed. Macroscopic examination revealed a 4 cm, well-circumscribed, tan-brown tumor, associated with diffuse adrenocortical hyperplasia. Histological examination confirmed the diagnosis of pheochromocytoma, without signs of aggressiveness. The tumor cells were immunopositive for Chromogranin A, Synaptophysin and ACTH. After surgery, catecholamine secretion returned quickly to normal level. Biological and clinical CS regression was noted. Conclusion: Despite the rare association of CS with pheochromocytoma, preoperative diagnosis is required to an appropriate therapy.