BACKGROUND Cerebellopontine angle lipoma is a rare tumor that composes less than 1% of all CPA tumors. There has been no recorded case of unilateral CPA/IAC lipoma with sudden contralateral deafness yet. METHODS We report a 52-year-old man diagnosed with right cerebellopontine angle lipoma and combined left total deafness. Pure-tone audiometry revealed total sensorineural deafness in his left ear and moderate sensorineural deafness in the right ear. The patient was treated with glucocorticoids, batroxobin, and other symptomatic treatments. There was no substantial improvement in hearing after 14 days' treatment. CONCLUSIONS We chose conservative treatment for him. It is advised to wear hearing aids in the right ear and to undergo regular imaging monitoring. CONCLUSIONS Treatment options for such patients should be chosen by taking into account the degree of bilateral hearing loss, the size and location of the tumor, the possibility of preserving hearing during surgery, the functional level of the patient's facial nerve, and other factors.