Abstract
Chronic or progressive ataxia in children may be caused by a number of conditions, including brain tumors, malformations and hereditary disorders. We report here a case of a 5 year old boy who presented with ataxia and developmental delay and on investigation was found to be a case of Joubert syndrome. MRI of the brain shows the characteristic molar tooth sign. Although, Joubert syndrome is not a common condition, yet it has clinical features which are very commonly found; that is why a high index of suspicion is needed, as such children are frequently misdiagnosed
