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Body dysmorphic disorder (BDD), characterized by a distressing or impairing preoccupation with nonexistent or slight defects in appearance, is associated with markedly poor quality of life and high rates of suicidality. Onset of BDD is usually in childhood or adolescence and, unless appropriately treated, tends to be chronic. The first-line pharmacologic approach for both delusional and non-delusional BDD is serotonin reuptake inhibitors (SRIs), often at high doses. SRI augmentation and switching strategies can be effective. The first-line psychotherapy is cognitive-behavioral therapy (CBT) tailored to BDD’s unique clinical features. Cosmetic treatment (such as surgery or dermatologic treatment), although received by a majority of patients with BDD, is not recommended. BDD has many similarities to obsessive-compulsive disorder (OCD) and appears closely related to OCD but also has some important differences. This article, which updates a 2015 article on BDD that we published in this journal, provides a clinically focused overview of BDD and its relationship to OCD.