Purpose of review: Since the last review of Williams syndrome in Current Opinion (2001) there have been many advances in knowledge about the cognitive, social and psychological impairments that characterize the disorder. The present review focuses on current research in these areas.

Recent findings: Williams syndrome is associated with a wide range of cognitive, linguistic, social and other difficulties. When young, these deficits may appear relatively mild – for example, many children are highly sociable and talkative – but with age the impact of these difficulties becomes more evident. Thus, inappropriate social behaviours can significantly increase the risk of social exclusion and vulnerability to abuse. Their superficially good speech can lead to educational and other services failing to understand the true extent of impairments or the need for specialist support. Mental health problems, especially related to anxiety, often become an increasing challenge from adolescence onwards.

Summary:The core difficulties associated with Williams syndrome have a cascading effect on many areas of development over time. However, specialist provision is rare and intervention trials are almost nonexistent. Longitudinal research is needed to identify factors associated with cognitive, social and emotional problems and to develop more effective ways of minimizing and treating difficulties.