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Anaplastic lymphoma kinase (ALK)–positive large B-cell lymphoma (ALK+ LBCL) is a rare, aggressive B-cell lymphoma that has earned its own classification in the most recent World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Here, we present an illustrative case of ALK+ LBCL and review the published literature, with an emphasis on the unusual characteristics of this tumor type that can easily lead to misdiagnosis. However, careful assessment of a combination of morphological and immunophenotypic features, as well as cytogenetic confirmation of an ALK rearrangement, can be used to reliably identify the entity in clinical practice. Although ALK+ LBCL is often poorly responsive to conventional chemotherapeutic regimens, the successes of ALK inhibitors in other ALK-expressing tumors raise the possibility of improved outcomes for patients with ALK+ LBCL with targeted therapy.