The association between mesotheliomas and ectopic ACTH secretion has been rarely reported; we present the first case of ectopic ACTH secretion (EAS) associated with a well-differentiated peritoneal mesothelioma in whom the high dose dexamethasone suppression test (HDDST) results and plasmatic ACTH levels were similar to those found in Cushing’s disease (CD). The gene discussed is POMC; the disease is peritoneal mesothelioma.