BACKGROUND: It is difficult to differentiate patients with cerebellopontine angle (CPA) cholesteatoma and patients with primary trigeminal neuralgia just according to early symptoms. We aimed to explore the clinical characteristics, early diagnosis, and microneurosurgical techniques for CPA cholesteatoma that presented as trigeminal neuralgia. METHODS: The data of 26 patients who complained trigeminal neuralgia with CPA cholesteatoma between January 2009 and December 2015 were collected and studied retrospectively, they were diagnosed by magnetic resonance imaging preoperatively and confirmed by pathology postoperatively. All the tumors were resected through a retrosigmoidal approach. In 26 cases, 14 patients who underwent cholesteatoma resection and microvascular decompression were assigned to group A and 12 patients who underwent only cholesteatoma resection were assigned to group B. The clinical features and surgical results between groups A and B were compared. The complications and surgical results were followed up, and surgical techniques were summarized. RESULTS: All patients presented as trigeminal neuralgia at the same side of the cholesteatoma. There was no statistical difference between the 2 groups in clinical features and surgical results between groups A and B. All patients with cholesteatoma showed clear and significant imaging characteristics. The tumors were totally removed in 18 patients and subtotally removed in 8 patients. Pain relief was satisfactory in all patients. Surgical complications included transient aseptic meningitis in 2 patients, facial numbness in 2 patients, mild tinnitus in 2 patients, mild and facial weakness in 1 patient. No death, hematoma, or acute hydrocephalus were reported in this series. During the follow-up of 12-80 months, no patient experienced recurrence of pain or tumor. CONCLUSIONS: Cholesteatoma of the cerebellopontine angle often presented as trigeminal neuralgia. Magnetic resonance imaging is helpful for early diagnosis according to its distinct signal. Surgical treatment is often the first choice, the neuralgia relief was satisfactory after operation. Microvascular decompression is recommended simultaneously if some offending vessels were founded during the surgical resection of the tumor.