Rationale:Neurilemmoma is a benign tumor derived from the Schwann cells of the nerve sheath. The highest incidence of neurilemmoma occurs in the head and neck region; however, the nose and paranasal sinuses are rarely involved. Less than 4% of these tumors involve the nasal cavity and paranasal sinuses. To date, only six cases of nasal vestibule neurilemmoma have been reported.Patient concerns:Two patients (a 32-year-old man and a 42-year-old woman) visited our clinic with complaint of a lump in the left nasal vestibule.Diagnosis:Histopathological examination and immunohistochemical staining confirmed a neurilemmoma.Interventions:The mass was completely removed via an intranasal approach.Outcomes and Lessons:Neurilemmoma is easy to overlook because it occurs rarely in the nasal vestibule, but neurilemmoma needs to be considered as a differential diagnosis.