&NA;A case of neurinoma affecting the intracranial part of the hypoglossal nerve is reported. Symptoms and signs, radiological findings, and operative results of other cases are reviewed. The value of computerized tomography and magnetic resonance imaging in local diagnosis and the feasibility of a one‐stage operation for total removal of tumors with extracranial extension are discussed (Neurosurgery24:583‐587, 1989)