Immune thrombocytopenia (IT) is a frequently occurring disease in childhood and a well known complication of HIV infection. Splenectomy is a part of the treatment strategy for severe chronic IT. However, overwhelming infections after splenectomy have limited its use, especially in young children. A 7-year-old child with maternal-fetal HIV-1 infection and related thrombocytopenia underwent splenectomy after previous treatment failed to improve her platelet count. Approximately 75% of the spleen was removed. The postoperative period was uncomplicated, and the platelet count increased significantly to greater than 500,000/mm3. Ultrasonographic examination performed 3 months later showed a stable volume of the spleen stump (40 x 40 x 20 mm) with effective "vascularization." The platelet count 12 months after surgery showed a sustained increase greater than 150,000 cell/mm3. Subtotal splenectomy may be a safe and effective alternative for patients with HIV and immune thrombocytopenia.