POMC and pheochromocytoma: Our clinical case outlines the diagnostic struggles, treatment challenges and surgical tactic for management of a rare ectopic ACTH producing pheochromocytoma.<h4>Discussion</h4>Highly variable clinical manifestations of ectopic ACTH producing pheochromocytoma, with typical signs of Cushing's syndrome and pheochromocytoma account for significant diagnostic difficulties and low incidence of verification of this pathology.