OBJECTIVE: To retrospectively analyze clinical data of 4 patients with trigeminal neuralgia (TN) secondary to osteoid osteoma (OO) of the petrous bone and discuss treatment for this rare disease. METHODS: Between January 2008 and December 2016, 4 patients in whom TN secondary to petrous bone OO was diagnosed received surgical treatment in Xuan Wu Hospital of Capital Medical University. We summarized the characteristics and treatments of this rare disease through retrospective review of the clinical information, imaging features, surgical details, and follow-up outcomes of the 4 patients. RESULTS: Among the 4 patients, 2 were men; mean age was 39.3 +/- 12.3 years (range, 26-52 years). All patients presented with symptoms of typical TN. Preoperative bone window computed tomography scan of the brain showed a regular bony lesion located at the petrous bone, contacting the trigeminal nerve. All patients experienced total relief of symptoms after surgical resection of the lesion by retrosigmoid sinus approach. During a mean follow-up of 14.2 months (range, 7-23 months), no preoperative symptoms recurred. CONCLUSIONS: TN secondary to petrous bone OO is extremely uncommon. Surgical decompression is an effective treatment for patients with petrous bone OO and associated TN.