We report the neuropsychological and MRI investigation of a patient (MV) who developed a selective impairment of visual-spatial working memory (WM) with preservation not only of verbal, but also of visual shape WM, following an ischemic lesion in the cerebral territory supplied by one of the terminal branches of the right anterior cerebral artery. MV was defective in visual-spatial WM whether the experimental procedure involved arm movement for target pointing or not. Also, in agreement with the role generally assigned to visual-spatial WM in visual imagery, MV was extremely slow in the mental rotation of visually and verbally presented objects. In striking contrast with the WM deficit, MV's visual-spatial long-term memory was intact. The behavioral and neuroanatomical investigation of MV provides support for the hypothesis that the superior frontal gyrus (BA 6) and the dorsomedial cortex of the parietal lobe (BA 7) are part of the neural circuitry underlying visual-spatial WM in humans.