A case of Mucha-Habermann disease (MHD), possibly associated with macrophage activation syndrome (MAS), is reported. The purpose of this paper was to describe the rare MHD (also known as pityriasis lichenoides et varioliformis acuta - PLEVA) in a 28-year-old male, who presented with generalized ulceronecrotic lesions on the skin and mucosae, gastrointestinal involvement, and heart and liver failure, associated with continuous high fever.The patient might have progressed to MAS and eventually died. The MHD is rare, potentially fatal and has severe systemic complications.The importance of early diagnosis and aggressive treatment is emphasized.