Atretic cephaloceles, which are a rare form of cranioschisis, are frequently associated with central nervous system (CNS) dysplasia. Here, we describe a case involving an atretic cephalocele without any CNS malformations. A parietal mass hadbeen observed on a 1-year-old boy since his birth. A physical examination revealed a 2-cm-round subscalp mass. A computed tomography scan revealed a defect of the skull at the parietal midline, and magnetic resonance imaging showed a T1-low- and T2-high-intensity mass that was connected to the dura without any malformation. The mass was surgically resected.Histologically, the mass comprised meningeal elements and fibrous tissue and did not include glial tissue. These findings suggested that it was a meningocele. An atretic cephalocele without CNS dysplasia has a good prognosis and can be treated by resection of the mass.