9-31 % of children with Down syndrome have atlantoaxial instability. These children might have the risk of atlantoaxial dislocation and spinal cord compression if they play sport or take part actively in daily Iife. The purpose of our study was to assess the presumed risk. We examined and followed up one year Iong 35 children with Down syndrome through a series of Iateral cervical spine x-ray and neurological examination. Those children with an atlantoaxial distance => 5mm were considered unstable. AlI children were allowed to continue their sport and daily activities. After one year the same examinations have been performed and it was found out that there was no statistical difference in the atlantoaxial distance. The neurological examinations were also normal. Because the children with Down syndrome are taking part in sport activities and daily Iife more often than before, the problem of atlantoaxial instability is gaining great importance. Our findings suggest that follow-up with routine Iateral cervical x-rays is insufficent and useless, beneath these there is no reason to restrict their daily Iives and to stop playing certain sport activities except those which are considered to be highly risky.