Anaplastic large cell lymphoma (ALCL), a pathologic entity of peripheral T-cell lymphomas, is one of the common non-Hodgkin's lymphomas in children. In the majority of childhood ALCL, they are characterized immunophenotypically by positive CD30 staining, and cytogenetically by ALK gene rearrangements with resultant oncogenic ALK fusion proteins. Nevertheless, ALCL is highly heterogeneous in terms of clinical manifestations, cytogenetic and molecular aberrations, therapeutic responses, and clinical outcomes. Specific ALCL histologies might be associated with characteristic immunophenotypic features, mutation profiling and gene expression signature, involving distinct signal transduction pathways. Integration of clinical relevant, tumor-associated and therapy-related risk factors help to improve ALCL risk stratification, to direct risk-based individualized therapy and to further improve prognosis of childhood ALCL. In the present paper, recent advances in this particular research field are reviewed.