Although acute myelogenous leukemia (AML) accounts for <20 % of leukemia in children, it is responsible for over half of all pediatric leukemia deaths. Improvement in event-free survival rates, now over 50 %, are due largely to intensification of chemotherapy, aggressive supportive care, development of risk stratification based on cytogenetic and molecular markers, and improved salvage regimens. Despite this improvement over the past few decades, the survival rates have recently plateaued, and further improvement will need to take into account advances in molecular characterization of AML, development of novel agents, and better understanding of host factors influencing toxicity and response to chemotherapy. This article reviews the epidemiology and biology trends in diagnosis and treatment of pediatric acute myelogenous leukemia.