The clinical and radiological features of 10 cases of congenital atlantoaxial dislocation have been studied. Ninety percent were male-The mean age at onset of symptoms was 9.3 years. Onset was gradual in 9 cases. The mean duration of symptoms was 3.3 years. A predisposing factor was seen in 150% cases. Progressive neurological deficit was the presenting symptoms in 7 patients while 3 had transitory attacks. Pyramidal involvement was seen in all. Occipitalisation, fusion of C2 and 3 and A.A. dislocation was seen in 50%. The dislocation was reducible in 30 percent. Surgery was done in 3 and 7 patients were treated conservatively.