BACKGROUND: Simultaneous presentation of multiple primary central nervous system (CNS) malignancies is extremely rare. There have been only eight cases of meningiomas co-existing with primary cerebral lymphoma, reported in the literature. CASE DESCRIPTION: We present a case of a patient who underwent surgical resection of an olfactory grove meningioma that was interdigitated with a primary CNS B-cell lymphoma. Following surgery, the patient was treated with high-dose methotrexate, and has no evidence of recurrence after 18 months. CONCLUSION: Because of the early recognition of these two distinct pathologies, the patient received directed adjuvant therapies, and has exceeded the survival of all other cases reported in the literature.