Acute myeloid leukemia is a heterogeneous group of malignant hematological disease characterized by replacement of normal bone marrow with abnormal, primitive hematopoietic cells. If untreated, it results with death, usually from bleeding or infection. It can appear at any age. Signs and symptoms of pediatric acute myelocytic leukemia (AML) can be divided into the: those due to the proliferation and infiltration of the abnormal leukemic cell population, those caused by a deficiency of normally functioning cells and constitutional symptoms. The disease usually begins insidiously, often in form of fever of unknown origin from which a child is not recovering, but rather remains pale and without will to play There are several types of acute myloid leukemia. Diagnosis includes a medical history and physical examination, blood tests, puncture bone marrow, immunophenotypic search and cytogenetic search. The most important are prognostic factors are age, presenting leukocyte count,immunophenotype, chromosomal abnormalities,presence or absence of disease in the central nervous system and response to initial therapy. Over the past decades, the therapy of pediatric acute myeloid leukemia has made remarkable progress. The long-term survival rate for pediatric patients with acute myeloid leukemia is nearly 60%. Acute myeloid leukemia accounts for about 35% of childhood deaths from leukemia. Mortality is a consequence of resistant progressive disease, relapse or treatment-related toxicity. Better understanding of the molecular mechanisms of leukemogenesis and new biologically-targeted therapies offer the reasonable promise of further improvement of treatment outcome in these children.