Acute myelogenous leukemia (AML) accounts for about 20% of the acute leukemias seen in children. In contrast to childhood acute lymphoblastic leukemia (ALL), there has only been a modest improvement in the cure rate of children with AML during the past two decades. Approximately 40% of children treated with chemotherapy alone are long-term survivors. The outcome is somewhat better for those children who are given bone marrow transplants from histocompatible sibling donors early in the first remission. During the last decade, however, new insights into the molecular basis of AML has increased our understanding of the pathogenesis and biology of this group of leukemias and are beginning to provide us with new therapeutic strategies.