BACKGROUND Primary aldosteronism and pheochromocytoma are endocrine causes of secondary arterial hypertension. The association of primary aldosteronism and pheochromocytoma is rare and the involved mechanisms are poorly understood. Either there is a coexistence of both diseases or the pheochromocytoma stimulates the production of aldosterone. Since management approaches may differ significantly, it is important to properly diagnose the 2 conditions. We describe concomitant pheochromocytoma and primary aldosteronism in a patient with resistant hypertension, which demanded a challenging and individualized approach. CASE REPORT A 64-year-old man was sent for observation in our department for type 2 diabetes and resistant hypertension. Laboratory work-up suggested a primary aldosteronism and a pheochromocytoma. The abdominal CT (before and after intravenous contrast, with portal and delayed phase acquisitions) revealed an indeterminate right adrenal lesion and 3 nodules in the left adrenal gland: 1 indeterminate and 2 compatible with adenomas. A 18F-FDOPA PET-CT showed increased uptake in the right adrenal gland. The patient underwent a right adrenalectomy and a pheochromocytoma was confirmed. An improvement in glycemic control was observed after surgery but the patient remained hypertensive. A captopril test confirmed the persistence of primary aldosteronism, and he was started on eplerenone, achieving blood pressure control. CONCLUSIONS This case highlights the challenges in diagnosing and treating the simultaneous occurrence of pheochromocytoma and primary aldosteronism. Our main goal was surgical removal of the pheochromocytoma due to the risk of an adrenergic crisis.