Hypoglossal neurinomas are rare tumors that present with gradually worsening unilateral wasting of the tongue. The tumors often attain large size prior to detection and often extend into the hypoglossal canal. Large tumors can distort the brainstem and cerebellum. Preoperative diagnosis can often be made by magnetic resonance imaging (MRI) with contrast enhancement. Traditionally, surgery for these tumors has entailed large transcondylar approaches, often necessitating occipitocervical stabilization. We managed one 23-year-old female patient with a large hypoglossal neurinoma. The tumor could be excised completely by a condyle-sparing minimally invasive suboccipital keyhole approach.