Congenital trichofolliculoma is a rare benign cutaneous tumor characterized by the presence of hair follicles in different stages of development within a dilated central follicular cavity.Veryrarely congenital cases have been reported.We present a case of fiveyearold girl with an asymptomatic hairy elevated lesion on her nose since birth. Laboratory investigations revealed normal complete blood picture,liver functionand renal function tests. Dermoscopy showed small tuft of hair protuding from centre of lesion. Skin biopsy confirmed the diagnosis of congenital trichofolliculoma. Simple surgical excision of the lesion was performed, resulting in complete healing without scarring. This case highlights the importance of recognizing and managing congenital trichofolliculoma in pediatric patients.