Febrile ulceronecrotic Mucha-Habermann disease in an 18-year-old man is reported. This disease is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) and is characterized by the sudden onset of diffuse coalescent ulcerations associated with high fever and systemic symptoms. In the present case the disease was preceded by typical PLEVA. Histologically, a leukocytoclastic vasculitis was seen in addition to the usual features of PLEVA. Findings of laboratory studies revealed an elevated erythrocyte sedimentation rate, a high white blood cell count, and a mild increase in liver enzymes. No systemic involvement was detected. Findings of T cell receptor gene analysis in skin and peripheral blood showed no abnormality. The patient was treated with PUVA and methotrexate with a good response. We review the eight previously reported cases of febrile ulceronecrotic Mucha-Habermann disease.