A 48-year-old man presented with symptoms consistent with Cushing's syndrome. Subsequent laboratory studies revealed markedly elevated adrenocorticotropic (ACTH) and cortisol levels, as well as a hypoklemic metabolic alkalosis. A pituitary MRI was performed, which revealed a normal pituitary; however, a large mass was seen centered in the ethmoid and paranasal sinuses with a significant amount of extension into surrounding structures. A biopsy was performed and pathology of the specimen was consistent with esthesioneuroblastoma. Immunohistochemical staining further defined the tumor as an ACTH-secreting esthesioneuroblastoma. After total resection of the mass and further treatment with adjuvant radiation therapy, the patient's symtoms completely resolved and the ACTH and cortisol levels were also greatly reduced. This case demonstrates the successful diagnosis and treatment of a rare neoplasm. Ectopic ACTH syndrome due to esthesioneuroblastoma is extremely uncommon with only five other cases being discussed in the literature.