In 10-30% of the patients with the 22q11 deletion syndrome (22q11DS), a psychosis develops in adulthood, often schizophrenia. 22q11DS is a common genetic syndrome which is associated with an interstitial deletion at chromosome 22q11. The syndrome is characterised by a variable phenotype which includes cognitive and behavioural problems, in addition to congenital heart and facial anomalies. The presence of 22q11DS represents one of the highest risk factors for the development of schizophrenia. The study of 22q11DS offers a unique opportunity to increase the understanding of the pathogenesis of schizophrenia.