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Hypoglossal Schwannomas are extremely rare benign slow-growing neoplasms, which originate from the 12th cranial nerve. To date, and to the best of our knowledge, only 40 cases of dumbbell-shaped Hypoglossal Swchannomas have been published in the world literature. We report our experience with a 66 years old male patient, who was diagnosed with a solido-cystic lesion at the right cerebello-pontine angle arising from XIIth cranial nerve. He was treated with surgery via midline suboccipital approach which led to sub-total removal of the tumor and improvement of the symptoms within 3 months. This case highlights the importance of an accurate suspicion diagnosis of hypoglossal schwannoma as well as the treatment options including surgery and radiosurgery.

The prognosis is nearly always good with a low risk of bleeding. A simple follow-up is often proposed because of the usual absence of complications.