Primary non-neoplastic aqueduetal stenosis associated with van Recklinghausen 's diease (neurofibromatosis) is a rare entity, aetiology of which is still unknown. About 19 similar cases have been reported in the literature, We report two additional cases presenting with this rare association. Our two patients had a good neurological recovery by ventriculo-peritoneal shunt procedure (follow up 4-6 months),