AbstractCongenital leukemia is a rare but well‐documented disease in which a leukemic process is detected at birth or very shortly thereafter. An estimated 175–200 reports of congenital leukemia have appeared in the literature. The majority of the cases reported have not undergone thorough immunophenotyping, but rather have been assigned lineage based on cytochemical and morphological studies. Historically, a large proportion of congenital leukemias have been thought to be of the myeloid lineage, in contrast to pediatric leukemias in general, which are primarily lymphoid. The precise proportions of the lineage assignments may be distorted by the inclusion of cases of transient myeloproliferative disorders (TMD) as congenital leukemia. The immunophenotyping data available to date suggest that congenital leukemias are phenotypically heterogeneous, lacking any common distinguishing markers. The prognosis for congenital leukemias is usually poor if leukemoid reactive processes, such as TMD, are carefully excluded. © 1995 Wiley‐Liss, Inc.