Background: Hypoglossal nerve schwannomas are uncommon, benign, and slow growing tumors arising from Schwann cells of the peripheral nerve sheath. They are well-documented in adult population. To the best of our knowledge, dumbbell-shaped hypoglossal schwannomas are rare and only 40 cases have been described in literature till date. They are managed surgically which poses signicant challenges including preservation of facial nerve. A 57 year old female presented with periodic spells of headache Case presentation: , imbalance while walking, trouble swallowing, right-sided tongue atrophy, and chronic chest infection. She was subsequently diagnosed with right cerebello-pontine (CP) angle hypoglossal schwannoma with extracranial extension. The tumor was managed by surgery and was performed in two stages: Stage one involved right retromastoid-sub occipital (RMSO) craniectomy with gross total excision of the lesion in the right CP angle followed by endoscopic assisted transoral-transpharyngeal excision of the tumor in the neck. Post-surgery, the patient showed gradual improvement. She could walk independently, her headache sub-sided and she showed slow improvement of lower cranial nerve function. However, during last follow up, it was observed that atrophy on the right side of the tongue still persisted. This case highlights t Conclusion: he importance of accurate diagnosis and treatment of hypoglossal schwannoma. Complete surgical excision is the recommended treatment for hypoglossal schwannoma which subsequently lowers the risk of recurrence and gives a good prognosis