Abstract: We report a 2-year-old boy with an elastic soft, flatly elevated, skin-colored nodule on his nasal ala. Histologic examination revealed numerous small hair follicles in several stages of maturation in the dermis. Serial sections did not show any cartilage or a central epithelial lined cystic structure. Based on clinico-pathologic findings, we diagnosed this lesion as a hair follicle nevus. Hair follicle nevus is quite rare. Histologically, it is very important not to find cartilage or a central epithelial lined cystic structure for distinction from an accessory auricle and from a trichofolliculoma, respectively. Awareness of the clinical and pathologic characterization of hair follicle nevus is an aid to a correct diagnosis.