Primary lesions of the hypoglossal canal, such as hypoglossal schwannomas, are rare. No consensus exists with regard to the surgical approach of choice for treatment of these lesions. Usually, lateral transcondylar approaches have been used. The authors describe the surgical anatomy of the midline subtonsillar approach to the hypoglossal canal. This approach includes a midline suboccipital craniotomy, dorsal opening of the foramen magnum and elevation of ipsilateral cerebellar tonsil to expose the hypoglossal nerve and its canal. The midline subtonsillar approach permits a straight primary intradural view to the hypoglossal canal. There is no necessity of condylar resections. The surgical anatomy of the subtonsillar approach is described and illustrated by an example of a case.