Congenital uterine anomalies are more common than previously recognized. While many women will have no symptoms or problems, some women with congenital uterine anomalies have increased risks of adverse outcomes during pregnancy. This article presents a case study of a woman with a congenital uterine anomaly leading to spontaneous rupture of her unscarred uterus remote from term. The most common types of congenital uterine anomalies and their associated reproductive risks are reviewed. Evaluation of congenital uterine anomalies and management alternatives are discussed.