Abstract: Anaplastic large cell lymphoma (ALCL) is a clinically rare non-Hodgkin’s lymphoma which is characterized by malignant “hallmark cells” and strong expression of CD30 in membrane and golgi patterns in almost all cases. The most frequent chromosomal translocation is t (2;5)(p23;q35). ALCL in children is nearly universally anaplastic lymphoma kinase (ALK) positive and commonly present with advanced systemic disease. Many different treatment strategies have been utilized with similar event free survival rates of 65%~75%. High-risk and recurrent/refractory ALCL can be treated by vinblastine weekly or hematopoietic stem cell transplantation. Novel therapies such as CD30 targeted treatment and ALK inhibitors may soon radically change the treatment paradigm for this disease. This review will provide an overview of the biological characteristics, clinical features, treatment and prognostic factors for children with ALCL.