Abstract： Objective To summerize the clinical characteristics and experiences of congenital encephalocele in children. Methods From May 1997 to May 2006, 48 cases of congenital encephalocele confirmed by surgical procedures and imageological studies were retrospectively analyzed. Results In this series, 28 cases were boys, 20 cases were girls, the age ranged from 1 day to 15 years. CT and MRI were the main means of diagnosis. The encephaloceles were located in occiput in 36, anterior cranium in 2, parietal region in 4 and basilar region in 6. Concomitan intracranial anomalies included gray matter heterotopia, callosal agenesis, septum pellucidum absence, hydrocephalus, Chiari malformation, Dandy-Walker syndrome and arachnoid cyst of cisterna magna. 3 of 48 cases did not undergo surgery. 4 cases with hydrocephalus received V-P shunts before or at the same time of encephalocele repair. 2 cases were put V-P shunts in time because of acute hydrocephalus after repair of encephalocele. Via nasendoscopy, 3 cases of basicranial encephaloceles underwent resection and repair. Conclusions Encephalocele is a congenital disease. MRI is the preferred diagnostic method and MR angiography is recommended simultaneously to clarify whether dural venous sinus protruded with the bulging tissues and to facilitate surgery.