BackgroundNeurofibromas (NF) are benign tumors of the peripheral nerves that are composed of Schwann cells, perineural-like cells and fibroblasts. The differential diagnosis for a solitary intraneural variant of neurofibroma arising in the floor of the mouth is broad and includes a submandibular gland neoplasm and adenopathy, among others. The intraoral approach is the best choice for a medium-sized lesion.Case presentationWe report a rare case of a solitary neurofibroma of the floor of the mouth in a 31-year-old male. The patient consulted the dental emergency department for acute pain of the left mandible. Systematic clinical examination revealed the presence of a mass in the left mouth floor. The panoramic x-ray was not conclusive and the magnetic resonance imaging (MRI) revealed a well-defined soft tissue lesion with homogenous isosignal intensity on the T1-weighted image, high intensity signal on the T2-weighted image and heterogeneous enhancement following contrast-enhancement on the T1-weighted Fast Sat image. The surgical excision of the soft-tissue neoplasm was accomplished by an intraoral approach. The specimen was sent for histopathologic analysis and Immunohistochemical studies which confirmed the diagnosis of a myxoid predominant intraneural solitary neurofibroma.ConclusionThe diagnosis of neurofibroma was confirmed by histopathological evaluation and immunohistochemical studies which also excluded other entities in the histopathologic differential diagnosis including schwannoma and a malignant peripheral nerve sheath tumor among other. Localized (solitary) neurofibromas most often occur as sporadic lesions, however; diagnosis of a solitary neurofibroma prompts clinical evaluation to exclude the remote possibility of neurofibromatosis. The purpose of this case report is to raise awareness of the uncommon presentation of neurofibroma and to document the successful management of such a lesion using an intraoral approach.