We describe 4 male children infected by the human immunodeficiency virus (HIV) who developed Burkitt's lymphoma during their disease. The clinical picture was characterized by an insidious appearance of symptoms. All the children suffered for several months from abdominal discomfort and a gradual elevation of their blood lactic dehydrogenase (LDH) level prior to diagnosis. Bone marrow involvement was found in 2 of the patients and jaw involvement in the other 2. After confirming the diagnosis of Burkitt's lymphoma, they were treated according to conventional protocols, with no need to reduce the dose intensity. They all went into complete remission and did not suffer from major opportunistic infections during chemotherapy. None of them relapsed. Two patients died from opportunistic infections 1 and 3 years after diagnosis. The other 2 are alive, 7 years and 6 months after diagnosis. The various characteristics of this unique pediatric group are described and the comparison of the clinical picture in adults is made, together with a review of the relevant literature.