Background: Intracerebral schwannoma is an extremely rare disease, accounting for fewer than 1% of intracranial schwannomas. The most common site for this type of schwannoma is the cerebral hemisphere, especially the frontal and temporal lobes; brainstem schwannoma is infrequent. Case Description: Here, we report a 51-year-old man with a monthlong history of blurred vision and weakness in his left lower limb. Magnetic resonance imaging showed a heterogeneous cystic tumor with a solid nodule arising from the brainstem. The patient underwent a craniotomy with complete resection of the tumor, which was confirmed to be a brainstem schwannoma by histopathological examination. We also performed a literature review of the 19 reported cases of brainstem schwannoma. Conclusions: Brainstem schwannomas predominated in children and young adults (60% of cases occurred in patients ≤ 30 years of age), and were more common in females (65%). Most of these schwannomas exhibited heterogeneous intensity, containing cystic (78%) and solid-enhanced components. The vast majority of reported cases (94.9%) followed a benign course, with an improved prognosis following tumor resection.