Williams syndrome (WS) is characterised by a defined genetic aetiology and a specific cognitive profile. It provides an opportunity to examine associations between neuroanatomy, behaviour, and genetics. High-resolution T1-weighted MRI of the brain of 12 patients with WS and 12 normal control subjects were used to estimate the shape and volume of the corpus callosum (CC), as well as the voxel intensity values as a measure its water content. The CC of patients with WS was more convex than that of normal control subjects and overall smaller in volume, particularly in the splenium and in the caudal part of the callosal body. In addition, there were higher ratio values of voxel intensity (i.e. less water content) in the mid-section of the body and the caudal part of the body of the CC. These combined features indicate an aberrant development of the CC in patients with WS and document some of the anatomical abnormalities that may underlie some of the cognitive impairments observed in subjects with WS.