Keratoacanthoma (KA) is a rapidly growing, low-grade neoplasm of pilo-sebaceous and hair follicle units which most often appears on the sun-exposed skin of the middle aged and older persons with multiple or localized occurrence. This tumor is dome-shaped nodule with a central keratinous plug. The etiology of this tumor is not obvious. Exposure to excessive sunlight is the most frequently noted responsible factor in the etiology of KA. About 80% of the tumors occur on the face. The histological features of the KA are often very similar to those of a cutaneous squamous cell carcinoma; however, the tumor structure usually provides a basis for their difference. There are many unusual cases of keratoacanthoma reported regarding site, size or other specifications. In this study, we excised a mass of nasal vestibule, a site far away sun-exposure. To our knowledge, this is the first case of nasal vestibular keratoacanthoma. For a clinician and a pathologist it is important to consider a benign lesion like Keratoacanthoma (KA) in the differential diagnosis of ulcerated nasal lesions and pay attention to differ it from Squamous Cell Carcinoma (SCC) which has a different and aggressive management.