Obsessive-compulsive disorder is a common mental disorder known to cause severe impairment across a person’s life span. However, to the best of our knowledge, this is the first study reporting an increased mortality risk among persons with OCD. We have found an increased risk of death by both natural and unnatural causes among persons with OCD. The co-occurrence of anxiety disorders or depression and substance use disorders resulted in a further increased risk of death. We found no familial confounding in mortality among persons with OCD, strengthening the hypothesis that OCD lies on the causal pathway to premature death. These findings represent an important first step toward identifying the underlying mechanisms for the risk of premature death among persons with OCD and stress the importance of using a thorough diagnostic process to identify comorbidities and accompanying problems. Moreover, our results emphasize that a focused effort on improving long-term outcomes among persons with OCD, including targeted treatment and suicide prevention, is essential.