Body dysmorphic disorder (BDD) is an often severe and disabling condition, affecting up to 2% of the population. Despite its prevalence and clinical significance, very little is known about the pathophysiology of BDD. However, clues to its possible neurobiological substrates and abnormalities in information processing are starting to emerge. This article reviews findings from genetic, brain lesion, neuroimaging, neuropsychological, and psychopharmacological studies that have allowed us to develop a tentative model of the functional neuroanatomy of BDD. There is likely a complex interplay of dysfunctions in several brain networks underlying the pathophysiology of BDD. A combination of dysfunctions in frontal-subcortical circuits, temporal, parietal, and limbic structures, and possibly involving hemispheric imbalances in information processing, may produce both the characteristic symptoms and neurocognitive deficits seen in BDD. An improved understanding of the pathophysiology of BDD will be crucial to guide the development of better treatments.