Six cases of congenital subscalp nodule associated with underlying cranium bifidum are reported. A plain skull roentgenogram showed a midline bone defect in the parieto-occipital region near the lambda. CT scan demonstrated neither brain malformation nor ventricular deformity except for the high position of the straight sinus. Cerebral angiography revealed an elongation of the vein of Galen and anomalous upward course of the straight sinus. At surgery, the tumor was solid and connected to a cord which extended intracranially via the cranium bifidum and blended with thickened arachnoid membrane either on the dorsal aspect of the midbrain or at the surface of the anterior vermis. Histologically, the tumor consisted in all cases of arachnoid cells and fibrous tissue with immature glial cells in one case. Possible pathogenesis of these tumors could be a result of the fetal nuchal bleb.