Desmoplastic trichoepithelioma (DT) is a rare benign adnexal neoplasm considered to have follicular differentiation. It usually presents as an asymptomatic, firm, annular plaque with a raised border. The most common site of occurrence is the face, usually on the cheek. Females are more often affected than males and the age range of patients previously reported is 8-79 years. We present a case of congenital desmoplastic trichoepithelioma. A girl was born at term to a healthy mother after an uneventful pregnancy and was noted to have widespread erythematous plaques with milia-like lesions over the right scalp, face and neck, with some areas of atrophic scarring. Histology and immunohistochemistry of incisional biopsies of the lesions were consistent with a diagnosis of DT. To our knowledge, this is the first reported case of congenital DT.