Malignant pyoderma is a destructive, ulcerating skin disease that occurs chiefly in young adults. Only eight cases of this rare disease have been reported. The head and neck have been involved in all cases, and a predilection for the periauricular region has been noted in several of the cases. Although the disease is progressive and chronic, responses to high-dose systemic corticosteroids have been noted, but usually a flare is associated with a reduction in dose. An additional case is described in which therapy with isotretinoin and sulfapyridine led to complete remission.