A 71-year-old male patient presented with malignant atrophic papulosis (Köhlmeier-Degos disease). He developed multiple distinctive cutaneous lesions. The histopathological findings showed an obliterated arteriole with a wedge-shaped area of ulcerated and necrobiotic dermis. Laboratory tests were within normal limits or not specific. Although the involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of the reported cases, in this patient so far the skin seems to be the only involved site. There is no proven effective therapy for Köhlmeier-Degos disease. In our case treatment with pentoxifylline and aspirin led to healing of all skin lesions within 3 months. One year after beginning treatment, the patient showed neither new cutaneous lesions, nor any signs of systemic involvement. The combination of pentoxifylline and aspirin should be considered when planning treatment strategies for malignant atrophic papulosis.