A 38-year-old HIV-positive man had several attacks of high fever associated with extensive perspiration over a 10-week period. Simultaneously, he developed molluscum contagiosum-like papules and an erythematous plaque on the face, ulcerated papules on both shoulders and buttocks and subcutaneous nodules on the arms. Histological examination of biopsy specimens revealed a diffuse, histiocytic infiltrate with abundant rod-shaped bacteria. Mycobacterium avium complex was cultured from the tissue and Mycobacterium avium complex DNA was detected by the polymerase chain reaction. The diagnosis of disseminated disease was additionally confirmed by culturing Mycobacterium avium complex from blood, sputum and stool. The skin lesions healed completely within 10 weeks by a multiagent as the patient was treated with a drug therapy. We describe the differential diagnosis, diagnostic procedures and therapy of disseminated infection with Mycobacterium avium complex.