Congenital sinuses of the upper lip are extremely rare anomaly. Only 17 cases have been reported in English literature. They were described as a pit which was the opening of a blind sinus, sometimes penetrated the orbicularis oris muscle, but had no connection with the oral cavity. The pit was reportedly located in a lateral or midline area. There are a few theories regarding the etiology of this rare anomaly, but the exact cause still remains obscure. We report a 4-year-old boy with congenital midline sinus of the upper lip. The sinus had a pit-like opening in the midline philtral area with a duct extending into 14 mm posteroinferiorly. It was excised by extraoral elliptical excision. Histopathological examination showed that duct was covered with stratified squamous epithelium.