Behçet disease (BD) is a rare, multifactorial condition with multiple manifestations affecting various systems. Bipolar disorder has been reported as more frequent in individuals with BD than in the general population. We report a case of BD and bipolar disorder and summarize previous findings in the literature concerning this topic. Our case illustrates the relevance of managing comorbid BD and bipolar disorder. It is crucial to manage both conditions to maximize the patient’s quality of life. Clinicians should be aware of the risk of bipolar disorder in patients with BD and vice versa and an integrated model of care could be useful. The exact biological and psychological aspects mediating the association between the 2 diagnoses remain unclear. Further, larger, prospective studies could help better clarify this relationship, leading to new advances in diagnosis and treatment.