Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL) develops to younger patients and is considered as a disease with better prognosis than other peripheral T cell lymphomas.[1] However, in an extremely rare subset of patients (3–4%), ALK+ ALCL with leukemic presentations is known to be chemotherapy-resistant.[1–3] Novel therapies, such as Brentuximab Vedotin (BV),[4,5] which is an anti-CD30 monoclonal antibody conjugated with monomethyl auristatin E, and ALK inhibitors,[6–11] have been tested for relapsed/refractory ALK+ ALCL. Here, ALK is linked to anaplastic large cell lymphoma.