INTRODUCTION: There is a paucity of published studies on the management and outcome of AIDS-associated Kaposi’s sarcoma (AAKS) in African children. In this study, we reviewed the management and literature of AAKS in Nigerian children. PATIENTS AND METHODS: A prospective review of children aged 1 to 14 years and adolescents aged 15 to 18 years who presented with AAKS. Following clinical evaluation and resuscitation, patients were treated with highly active antiretroviral therapy (HAART). Stable patients were further treated with chemotherapy consisting of vincristine, doxorubicin, and bleomycin. Patients were monitored until death or loss to follow-up. RESULTS: There were 9 patients: 6 children and 3 adolescents. Three children had vertical transmission of HIV infection. Kaposi’s sarcoma was the AIDS-defining disease in 5 patients. One patient was on HAART at the time of diagnosis. There were multiple skin lesions in all patients, and cervical lymph nodes and oropharynx were frequently affected. The CD4 counts at the time of AAKS diagnosis ranged 78 to 601 cells/ uL, mean of 317. Five patients had best palliative care. Three had anticancer chemotherapy, of which 2 were alive 4 years after diagnosis. Three patients died at the initial hospitalization 2 to 6 weeks after diagnosis. CONCLUSION: Children and adolescents with AAKS presented with generalized skin lesions and lymphadenopathy, which facilitated the diagnosis. The majority of the patients presented with advanced disease that was rapidly fatal. However, patients with good immunity may have a prolonged control of symptoms if treated with HAART and appropriate anticancer chemotherapy.