Malignant atrophic papulosis (Degosdisease) was first described in detail by Degos and his co-worker in I942, In this rare disease of unknown etiology, porcehun-white skin lesians develop, and death usually occurs from perforation of the intestine weeks to years after onset, and also but rarely from infarction of central nervous system. Our case of Degos disease in an 18 years old man with typical skin lesions and abdominal symptoms such as pain, nausea and distension due to gastrointestinal involvement is described. He died of peritonitis 9 months after onset. The biopsy specimen of an atrophic papule showed thrombotic occlusion of an arteriole. The authors experienced a case of Degos disease diagnosed by clinical and histopathologieal findings for the first time in Korea.