Neuromuscular choristoma (NMC) is an uncommon tumor that usually involves a large nerve trunk. Only 28 cases of NMC have been previously reported in the English-language literature, 17 of which involved cranial nerves. We report a new case of intracranial NMC that arose from a facial nerve at the cerebellopontine angle in a 44-year-old man. The patient was taken to surgery, where the lesion was found to involve the right facial nerve. The tumor was partially removed, and at the 2-year follow-up, the patient showed no sign of recurrence.