Objectives: This systematic review summarizes all published uterine hemangioma cases in pregnant women. Search strategy: The databases PubMed and Scopus were searched systematically. The reference lists of all retrieved articles were also screened. Selection criteria: The online tool Rayyan QCRI was used for registration of the selection process. Articles reporting on cases of uterine hemangioma in pregnancy were included, non-English articles were excluded. Data collection and analysis: Data extraction was done by one reviewer and thereafter verified by the second reviewer. All data were described in a narrative format. Results: Fifteen case reports were included. In most cases, the diagnosis was established by antenatal ultrasound and pregnancy course was uneventful. More than half of the patients developed a postpartum hemorrhage, necessitating a hysterectomy for bleeding control in four cases, although the risk for both seemed lower in those patients in whom the hemangioma was diagnosed before delivery. One case of maternal mortality and two cases of fetal death were reported. There was one case of neonatal respiratory morbidity, although the neonatal data were not routinely reported upon. Conclusion: Current knowledge on uterine hemangioma in pregnancy is limited, but it seems to hold substantial risks for both mother and child. We recommend routine screening for this condition at the standard mid-trimester anomaly scan. Pregnant women with uterine hemangioma should ideally be cared for in centers of expertise. An international registry will help to build a better understanding of this rare pathology. Funding: None.