The authors report three cases of primary nontumoral aqueductal stenosis associated with von Recklinghausen's disease in children. Moreover, 16 similar cases collected from the literature are presented. The clinical features are evaluated in light of data from the literature. Among all 19 cases, the median age was 19 years (range 6-46 years), and nine patients were under the age of 13 years. Our own patients underwent ventriculoatrial shunt procedures, followed by clinical recovery (follow-up 2-5 years). In one patient, computed tomography scanning performed 20 months after the operation showed a chronic calcified subdural hematoma in the right frontoparietal area. In conclusion, it is felt that, among all the pathological events usually described in patients affected by neurofibromatosis, primary aqueductal stenosis seems to be part of the natural history of this neurological syndrome.