Despite its prevalence and clinical significance, very little is known about the neurobiology of body dysmorphic disorder (BDD). However, information about the possible neurobiologic substrates of BDD is starting to emerge. Family and genetic studies suggest that BDD is a hereditary disorder. There is indirect psychopharmacologic evidence implicating serotonin in modulating BDD symptoms. Neuropsychological studies demonstrate abnormalities in memory, executive functioning, emotion recognition, and visual self-perception. Case studies of BDD symptoms resulting from brain damage implicate right temporal regions. Studies of the neural basis of body image distortion suggest both involvement of the right parahippocampal gyms in appraisals of body image, and dysfunction of specific temporal and parietal cortical regions in mediating distorted visual self-perception. Reactions of disgust and anxiety to visual perceptions, which are fundamental processes in BDD patients, may be due to excessive activation of the insula and amygdala. The neurobiologic basis of BDD is likely a complex interplay of dysfunctions in several neural systems. A combination of dysfunctions in frontal-subcortical circuits, right temporal and parietal cortical areas, and limbic structures may produce both the symptoms and neurocognitive deficits characteristic of BDD.