| Summary: | Neurofibromas are common nerve sheath tumors, occurring either sporadically or associated with Von Recklinghausen’s disease. Only 6.5% of solitary lesions are seen to involve the oral cavity without any features of neurofibromatosis type 1 (NF-1). It presents as a soft, skin-colored nodule with a characteristic buttonhole invagination. Histologically, it is an unencapsulated lesion consisting of proliferated neural elements, with a background of mucin and mast cells. Surgical excision is the treatment of choice. A 49-year-old female patient presented with a 35-year history of single, asymptomatic lesion over the lower lip, with no features of NF-1. On the basis of the history, histopathological findings, and dermoscopy, a diagnosis of neurofibroma was made and the lesion was excised, with no recurrence over a period of 1 year. The present case report has been reported for its interesting presentation and unusual site of involvement.
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