Conclusion: To our knowledge, this is the first description of a malignant adrenal tumour in the setting of AHF. As illustrated by this case, awareness of AHF as an entity and attention to its distinctive gross and histologic features are essential to avoid confusion between adrenal and hepatic lesions, especially when imaging studies have provided misleading findings. Objective: Cushing's syndrome (CS) is a rare disease, resulting in the majority of cases from ACTH hypersecretion. 70 % is of pituitary origin, 20 % of adrenal origin, and only 10 % from ectopic ACTH production. Method: We reported a rare cause of ectopic ACTH dependent CS, caused by a pheochromocytoma.