Severe disseminated infection due to Mycobacterium avium intracellulare, with unusual cutaneous features, is reported in a patient with acquired immunodeficiency syndrome (AIDS). The eruption appeared as disseminated pustular lesions which showed necrotic features and which led to varioliform scarring. Bacterial culture from the skin, blood, and bone marrow, and ultimately from the bronchoalveolar fluid and sputum, was positive for M. avium intracellulare. The patient was successfully treated using a multiple agent anti-mycobacterial regimen including clarithromycin, which appeared to be the most effective drug. This resulted in resolution of the cutaneous and general symptoms. Our patient illustrates the wide spectrum of skin presentations that may be seen with mycobacterial infections in subjects infected with the human immunodeficiency virus (HIV). Clarithromycin is an important agent for the treatment of these severe infections.