Velo-cardio-facial syndrome (VCFS) is acongenital malformation syndrome of vari-able clinical expression. The syndrome s major features are cleft palate, cardiac anomalies, characteristic facies, and learning disabilities. Recently, psychiatric disorders have been reported in over 10% of patients with VCFS. We report a schizophrenic woman with features of VCFS and detection of a 22q11 deletion. This observation suggests that VCFS and certain psychotic disorders may share a similar pathogenic mechanism.