A variety of diseases can affect the nasal vestibule. It might be challenging to diagnose and treat a nasal vestibular tumor due to the anatomical characteristics of the nasal vestibule. Neurilemmoma is a tumor derived from Schwann cells of the nerve sheath. Less than 4% of these tumors invade the nasal cavity and sinuses. Nasal vestibule neurilemmoma is rare, it is often overlooked when a mass discovered. The diagnosis of it is mainly based on clinical symptoms, nasal endoscopy, and imaging, The mainstay of treatment is complete resection surgery. Pathological examination provides the final diagnosis. We present a patient with nasal vestibule neurilemmoma who underwent a successful endoscopic surgery without cosmetic deformity, and discuss the clinical manifestations, histological features, imaging features, differential diagnosis, treatment options, then reviewed relevant literature of this rare benign lesion.