A 62-year-old man was admitted to our hospital complaining of dysphagia and hoarseness that had persisted for five days. A neurological examination indicated bulbar palsy. Brain magnetic resonance imaging showed thickening of cranial nerves IX, X and XI, in addition to pineal body enlargement with diffuse contrast enhancement. A tumor biopsy overriding the spinal root of the right XIth cranial nerve was performed. The histologic analysis confirmed a diagnosis of diffuse large B-cell lymphoma. Malignant lymphoma should be considered in the differential diagnosis of pineal region tumors. Furthermore, obtaining histological confirmation is crucial for making proper management decisions.