Abstract: Anaplastic lymphoma kinase-positive(ALK⁺) diffuse large B-cell lymphoma(DLBCL) is a rare subtype of DLBCL.ALK⁺DLBCL has a characteristic immunoblastic/plasmablastic morphology, a distinct immunophenotypic profile, and recurrent cytogenetic/molecular genetic abnormalities.The occurrence of this type of lymphoma has been reported in both adult and pediatric populations.Although rare, this new entity should be recognized because most cases follow an aggressive clinical course with a poor prognosis.The response of ALK⁺DLBCL to conventional chemotherapy is poor.The recently discovered small molecule ALK inhibitor may provide a potential therapeutic option for patients with this disease.