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We report a case of atretic cephaloce in a 7-month-old girl who presented with a growing left parietal painful mass, which was covered with excessive growth of thick scalp hairs and purplish discolored skin. Preoperative radiological studies revealed the left parietal round skull defect and epidural well enhancing mass without significant intradural parenchymal and vascular malformation. Total excision was done and histological diagnosis of atretic cephalocele was done. Characteristic histopathological features of this rare congenital anomaly with pertinent review of literatures were presented. Key words:Atretic cephalocele;Congenital anomaly;Scalp mass;Pariental skull defect.