We present a case of sarcoidosis with severe autonomic dysfunction. A 51 year-old male was diagnosed as having sarcoidosis based on the findings of uveitis, bilateral hilar lymphadenopathy and elevated serum angiotensin converting enzyme. He was admitted to our department because of numbness of the trunk and limbs as well as frequent episodes of orthostatic syncope. Neurological findings were proximal dominant muscle weakness and atrophy of the limbs, dysesthesia of the hands and trunk, paresthesia of the anterior thighs, decreased vibratory sensation, and autonomic dysfunction such as orthostatic hypotension, constipation and impotence. A skin biopsy of the plaques revealed epithelioid granulomas and giant cells. Nerve biopsy showed decreased density of the unmyelinated fibers with axonal degeneration. These findings, along with abnormal autonomic function tests, suggested pan-autonomic failure with impairments of post ganglionic sympathetic nerves. Though sarcoidosis with autonomic dysfunction has rarely been reported, sarcoid neuropathy may warrant further investigation of autonomic functions., 神経内科学教室岩田誠教授退任記念特別号