Schwannomas are benign neurogenic tumors that arise from the perineural Schwann cells of nerve sheaths. They are extremely rare and usually present as solitary, encapsulated lesions. We report a rare case of schwannoma on the upper lip and discuss the clinical presentation, diagnosis, and treatment approaches. A 19-year-old female patient presented with swelling of 6 months duration on the upper lip. Physical examination revealed a mobile mass with elastic consistency at the junction of the upper lip and nasal base, which was approximately 3 cm in diameter. Imaging studies revealed an encapsulated mass limited to the upper lip with sharp contours. With a preliminary diagnosis of salivary gland tumor, the mass was completely excised under general anesthesia by blunt dissection through a sublabial incision. Histopathological examination of the tumor reported the lesion as a schwannoma. Although there are few such cases in the literature, clinicians should consider the possibility of this rare tumor in the differential diagnosis of tumors localized in the lip region. (C) 2015 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.