OBJECTIVE To study the epidemiologic and clinical features of infection with Mycobacterium tuberculosis in human immunodeficiency virus (HIV)-infected children and their families. PATIENTS AND CLINICAL SETTING: Sixty families of children with HIV infection, children of HIV indeterminate status, and seroreverters underwent follow-up in a comprehensive multidisciplinary program for children and families. METHODS Infection with M tuberculosis was diagnosed based on a positive Mantoux test result or a positive culture. RESULTS Mycobacterium tuberculosis infection was diagnosed in seven children (three infected with HIV, three seroreverters, and one uninfected sibling of an infected child) from four families (6%). All infections were detected in the period from March 1990 through January 1992. Six of seven children had a history of exposure to M tuberculosis in an HIV-infected adult (parent) who was an intravenous drug user, homeless, and/or noncompliant with the medical regimen. All HIV-infected children and one seroreverter had pulmonary tuberculosis. One child died of complications of tuberculosis and HIV infection. The M tuberculosis isolated from this child was resistant to isoniazid, rifampin, and streptomycin sulfate. CONCLUSIONS Tuberculosis is a growing problem among inner-city children born to HIV-infected parents. Children infected with HIV in this study had symptomatic and severe disease with tuberculosis, which reflected the drug susceptibility pattern of M tuberculosis seen in our community.