Central nervous system (CNS) involvement by Hodgkin lymphoma (HL) is rare. As a result, there is limited guidance for clinicians on how to manage these patients. Detailed information was collected on 16 patients, the largest number to date, with meningeal or parenchymal CNS-HL confirmed by histopathology (15) or CSF (1). Eight patients presented with CNS-HL at diagnosis, 2 of whom had isolated CNS disease, while 8 patients developed CNS-HL at relapse. Patients received a range of treatments including surgery or radiation alone, radiation with chemotherapy, or chemotherapy alone. Median overall survival for all 16 patients was 60.9 months from first diagnosis of HL (systemic or CNS) and 43.8 months from diagnosis of CNS-HL. Although a majority of patients have died, long-term survival is possible in patients who achieve a complete response to treatment, particularly those who present with CNS involvement or involvement of the CNS is the sole site of relapsed disease.