Importance: Spontaneous perinatal rupture of a uterine vessel is a rare occurrence that may lead to severe hemorrhage and requires prompt identification and management.Objective: The aim of this study was to examine the etiologies, locations, diagnostic tools, treatment options, and risks in subsequent pregnancies when spontaneous rupture of a uterine vessel occurs in pregnancy.Evidence Acquisition: A literature search was performed by university research librarians using the PubMed, CINAHL, and Web of Science search engines. Identified were 78 cases of perinatal spontaneous uterine vessel rupture and formed the basis for this review.Results: Increased uterine blood flow during pregnancy may alter the integrity of pelvic vessels leading to increased risk of spontaneous rupture. The uterine artery is the most common site of vessel rupture; the second most common site is the uterine-ovarian plexus. The most common presentation is abdominal or pelvic pain, maternal vital sign abnormalities, and an absence of vaginal bleeding. Exploratory laparotomy and embolization (interventional radiology) have been reported as management options.Conclusions: Spontaneous rupture of uterine vessels is a rare but potentially life-threatening complication of pregnancy that should be included in the differential diagnosis of pregnant patients presenting with an acute abdomen.Relevance: Our aim is to increase the awareness of spontaneous vessel rupture during pregnancy to improve detection, management, and perinatal outcomes.Target Audience: Obstetrician-gynecologists, radiologists, family medicine physicians Learning Objectives: After completing this CME activity, participants should be better able to describe spontaneous uterine vessel rupture; identify its risk factors and presenting symptoms; and explain preferred diagnostic imaging modalities, management options, and possible complications in future pregnancies.All authors, faculty, and staff have no relevant financial relationships with any ineligible organizations regarding this educational activity.