Anaplastic large cell lymphoma (ALCL) is a rare, high-grade, T-cell neoplasm classified into cutaneous primary, systemic primary ALK-positive (ALK+), systemic primary ALK-negative, or breast-implant associated. Secondary involvement of the central nervous system (CNS) by systemic primary ALK+ ALCL is a rare occurrence. We present a case of CNS involvement by ALK+ ALCL eleven years after diagnosis of the primary tumor in the thoracic vertebra. The anatomopathological examination confirmed the diagnosis of ALK+ ALCL. A brief review of the treatment and the clinical and pathological aspects is presented.