In the central nervous system, cerebellopontine angle (CPA) lymphomas are rare; few cases have been reported. Lymphoplasmacytic lymphoma (LPL) in the CPA is rarer still, and often misdiagnosed as acoustic neuroma.We report a rare case of CPA LPL-a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological test.A 43-year-old woman presented with headaches. Her magnetic resonance imaging revealed an abnormal homogeneously enhancing mass in the left CPA. We present detailed analysis of her disease and review relevant literature.When surgically treated, her specimen showed a typical LPL histopathology pattern. After surgery, the patient's symptoms improved greatly, and she received chemotherapy.Despite its rarity, LPL should be considered in differential diagnoses of CPA lesions that mimic acoustic neuromas.