Abstract
Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) has a high relapse rate. However, it typically remains confined to the skin and has a favorable long-term prognosis. We describe a case of PC-ALCL that experienced a relapse in the central nervous system (CNS). The patient presented with somatosensory abnormalities in the extremities after local treatment of skin lesions and was diagnosed with CNS relapse of PC-ALCL. Methotrexate, procarbazine, and vincristine therapy, and alternating brentuximab vedotin, followed by autologous hematopoietic stem cell transplantation (ASCT) cured the CNS lesions, whereas the skin lesions relapsed early. PC-ALCL could relapse in the CNS; systemic chemotherapy and ASCT may be effective.