Mycobacterium avium-intracellulare (MAI) is a ubiquitous organism with limited virulence in the immunocompetent host. Disseminated disease is associated with a high mortality rate. Except for localized cervical adenitis, MAI disease is rare in immunocompetent children. We report a child with antibody deficiency (dysgammaglobulinemia) and disseminated MAI infection, in whom complete, long-term remission was attained with multiple antimycobacterial therapy. The patient presented with progressive cervical lymphadenopathy and hepatomegaly at 7 years of age. A lymph node biopsy showed acid-fast bacilli and granulomas. Despite a transient response to conventional antituberculous therapy, including isoniazid and rifampin, his symptoms progressed. Cultures from blood, bone marrow, spleen, and cervical lymph node tissues revealed an MAI organism. Subsequent treatment using a combination of clarithromycin, amikacin, and ethambutol for 16 months resolved clinical symptoms, and subsequent blood culture results became negative. By the time of this report, the patient has been disease-free for 4 years. Multiple-drug therapy is promising for the treatment of MAI in children with antibody deficiency; however, the selection of antiinfective drugs should include a member of the newer macrolide family. acquired immunodeficiency syndrome, clarithromycin, dysgammaglobulinemia, Mycobacterium avium-intracellulare, treatment.