Neurodevelopmental factors have been implicated in the pathophysiology of mental disorders. However, the evidence regarding their role in bipolar disorder is controversial. We reviewed the pertinent literature searching for evidence regarding a neurodevelopmental origin of bipolar disorder. Findings from clinical, epidemiological, neuroimaging, and post-mortem studies are discussed, as well as the implications of the available data for a better understanding of the mechanisms involved in the genesis of bipolar disorder. While some evidence exists for developmental risk factors in bipolar disorder, further research is needed to determine the precise extent of their contribution to pathogenesis. The timing and course of such developmentally mediated neurobiological alterations also need to be determined. Of particular importance for further study is the possibility that bipolar disorder may be mediated by an abnormal maturation of brain structures involved in affect regulation.