RESUMO
INTRODUCTION: Cases of ectopic production of adrenocorticotropic hormone are considerably rare, but persistent in clinical practice. Extremely rare cases of ectopic production, such as via pheochromocytoma secretion, require special clinical attention and prior knowledge. It is important to understand the diagnostic algorithm for identifying ectopic sources of adrenocorticotropic hormone production. PRESENTATION OF CASE: In this clinical vignette we report a patient with a complex variety of clinical symptoms and no discernable cause for hypercriticism. Our clinical case outlines the diagnostic struggles, treatment challenges and surgical tactic for management of a rare ectopic ACTH producing pheochromocytoma. DISCUSSION: 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. Correction of symptoms and patient stabilization are of utmost importance throughout treatment. CONCLUSION: ACTH-dependent Cushing's syndrome, caused by a pheochromocytoma is extremely rare, but should be considered as a possible source for ACTH production. The diagnostic challenges of this condition can be met with confidence when a strict search protocol is conducted for detection of ACTH source.