Central nervous system (CNS) involvement by Hodgkin lymphoma (HL) is extremely rare, accounting for 0.5% or less of HL cases. In contrast, CNS involvement can occur in 5-30% of patients with non-Hodgkin lymphoma. CNS HL can present at any point in the course of HL, most commonly during relapsing disease, and has been described in both immunocompromised and immunocompetent patients. We describe a case of HL affecting the CNS and bone marrow on initial presentation in a 79-year-old immunocompetent female with a prior history of squamous cell carcinoma of the larynx and adenocarcinoma of the lung. Following the case report, a review of the literature on CNS HL is presented.