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. Here, POMC is linked to hereditary pheochromocytoma-paraganglioma.