A case of peripheral carcinoid tumor of the lung associated with Cushing's syndrome is reported. This 31-years old man presented Cushing's syndrome 10 years ago and underwent bilateral adrenalectomy. Cushing's syndrome was relieved postoperatively but was followed by pronounced hyperpigmentation 8 years later. The lung nodule which was noticed at the 1st admission enlarged recently. Pneumonectomy specimen revealed gray a brown well demarcated mass in the periphery of the lung. Microscopically the tumor consisted mainly of spindle cell element. on electron microscopy membrane bound neurosecretory granules were identified. On PAP stain ACTH was demonstrated in the cytoplasm. In summary the peripheral carcinoid produced ectopic ACTH with resultant Cushing's syndrome and hyperpigmentation.