Introduction Schwannomas or neurilemmomas are well demarcated, benign neurogenic lesions arising by a fibroblastic proliferation of the nerve sheath cell (Schwann cell). They usually present as solitary encapsulated lesions with rare occurrence in the upper lip. Non-diagnosed or misdiagnosed schwannomas present a high risk for the tumor to continue growing and exerting pressure on surrounding nerves. These tumours based on their location could lead to facial weakness and paralysis, pressure in ears, tinnitus, hearing loss, balance loss, and could lead to a life-threatening situation. Case Outline This case is a rare presentation of a schwannoma located in the upper lip of a 21-year-old male patient of Indian origin. The patient complained of a swelling in the mouth with a difficulty in keeping the mouth closed. The swelling was surgically excised and the patient healed completely. Conclusion This case of occurrence of tumor on the upper lip points to the possibility of considering schwannoma as a possibility in the diagnosis of oral tumors in the future, as the location of the tumor was rare and had a high chance of misdiagnosis.
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