Introduction: Pheochromocytoma is an exceptional cause of Cushings syndrome. We report the very exceptional case of a composite pheochromocytoma-ganglioneuroma with ectopic secretion of adrenocorticotrophic hormone, two rare histological entities grouped into one, in a patient with a complex clinical feature. Case Report: This is a 62-year-old patient consulting for polyarthralgia and low back pain in whom the physical examination found Cushings syndrome. The thoraco-abdomino-pelvic computed tomography showed bilateral adrenal masses. A left adrenalectomy was performed first. Histological examination shows a double contingent tumor proliferation. The diagnosis of composite pheochromocytoma-ganglioneuroma with ectopic adrenocorticotrophic hormone secretion was retained. Discussion and Conclusion: We have not found any reported case of ectopic secretion of adrenocorticotrophic hormone linked to a composite pheochromocytoma-ganglioneuroma in the French and English literature during the last 10 years. The diagnosis is essentially histological because clinical and radiological data do not allow it to be differentiated from a classic pheochromocytoma. This entity highlights diagnostic and therapeutic difficulties.