We are reporting a case of acute myeloid leukemia in a child with pericardial effusion and cardiac tamponade, with a review of the literature. Morphologic and cytochemical evidence was used to diagnose acute myeloid leukemia, and radiologic, electrocardiographic, echocardiographic, cytologic, and histologic evidence confirmed the diagnosis of tamponade and pericardial leukemic involvement. Pericardiotomy and pericardial window drainage successfully relieved the tamponade, and conventional chemotherapy was used to induce a remission