A newborn baby with a leaking frontoethmoidal encephalocele is reported. The 1-day-old baby was referred to our center with a swelling on the nose leaking CSF. The child was the third baby of a consanguinous marriage. The baby had a swelling over the nose, 8 cm in diameter. The center of the swelling looked unhealthy, with a small raw area seeping CSF. CT scan showed a swelling containing tissue isodense with normal brain. The bone window and the 3D CT revealed a basal bone defect in front of the crista galii and an outer bone defect at the frontonasal junction over the nasion. The baby was operated on the 5th day after birth. One-stage repair of the encephalocele was performed, along with correction of hypertelorism and bone grafting over the forehead. The baby withstood the surgery well and was discharged from the hospital on the 10th postoperative day.