There has been an increase in neuroimaging research in body dysmorphic disorder (BDD), yet little is known about the underlying neurobiological basis of the disorder. We aimed to provide a systematic overview of the literature on the neurobiology of BDD. Two reviewers undertook a search of three electronic research databases: PubMed, PsycINFO, and Google Scholar. The search consisted of synonyms commonly associated with BDD and methods to evaluate brain structure, function, and network organisation. Out of an initial yield of 175 articles, 19 fulfilled inclusion criteria and were reviewed. We identified differences in brain activity, structure, and connectivity in BDD participants in frontostriatal, limbic, and visual system regions when compared to healthy control and other clinical groups. We put forth a neurobiological model of BDD pathophysiology that involves wide-spread disorganisation in neural networks involved in cognitive control and the interpretation of visual and emotional information. This review considers how this model might aid in the development of future research and understanding of BDD.