The etiology of occipital plagiocephaly (OP) is not fully understood. The authors have observed that many infants with this condition have external hydrocephalus. This study was undertaken to quantify cerebrospinal fluid (CSF) space caliber in children with OP and to compare these measurements to those derived from normal age-matched controls to further elucidate the pathogenesis of this condition. Thirty-one infants with isolated unilateral OP (mean age 6 months) were studied. Infants with multiple cranial suture abnormalities, impaired neurological function, developmental delay, and associated craniofacial anomalies were excluded. Twenty normal infants were evaluated as controls. The volumes of the sylvian fissures, frontal and occipital subarachnoid spaces, as well as the cross-sectional areas of the suprasellar and perimesencephalic cisterns, were calculated from computerized tomography (CT) studies. Ventricular size was also assessed. Generalized subarachnoid space dilation was observed in 29 (93.5%) of the 31 children with OP. Head circumference was significantly greater in the case group (71.4 vs. 50.8 percentile; p = 0.0002 by analysis of variance). The sylvian fissure volume was significantly larger in the case group (5.8 ml vs. 0.7 ml in controls, p < 0.0001). The volume of the contralateral sylvian fissure was greater than that ipsilateral to the side of OP (7.1 ml vs. 4.5 ml, p = 0.001). Frontal subarachnoid space volume was greater in infants with OP (27.5 ml vs. 0.6 ml in controls, p < 0.0001). Both the suprasellar and perimesencephalic cisterns were of greater caliber in the case group (p = 0.007 and p < 0.0001, respectively). No difference in ventricular size or occipital subarachnoid space volume was noted between groups. The extraventricular CSF spaces in neurologically unimpaired infants with OP are significantly larger than those in age- and sex-matched controls. Enlarged subarachnoid spaces may increase the compliance and malleability of the calvaria and sutures, predisposing to positional deformity. External hydrocephalus may be a fundamental etiological factor in OP.