Background: Anti-Rh17 is a rare red blood cell (RBC) antibody to high-frequency antigens that may cause severe hemolytic disease of the fetus and newborn (HDFN). Despite the rarity of HDFN caused by Anti-Rh17, this antibody was reported in many different populations. Emergency transfusions, especially exchange transfusions, present a huge problem if no compatible RBCs of phenotype D-- are available. Methods: Here we report obstetrical histories of three women and describe their pregnancies complicated by anti-Rh17 antibodies. We summarized published cases of pregnancies complicated by anti-Rh17 and reviewed transfusion treatment and outcomes. Additionally, a simplified flowchart for the management of such pregnancies is proposed. Results: Four pregnancies were affected by severe HDFN, and three of them ended with perinatal death. In the fourth case, the baby was born hydropic and icteric and the condition was rapidly deteriorating. Emergency exchange transfusion was performed with incompatible O-negative RBC units in AB-negative plasma. The baby was discharged on the 14th day in good health. In the available literature, 15 women and 22 pregnancies were reported, 20 of them developed severe HDFN. According to the data, intrauterine transfusion for treatment of HDFN was the most common form of treatment with the donation of the mother’s blood. Different options for exchange transfusion were described, including incompatible RBCs. Conclusion: In more than 90% of described pregnancies of HDFN caused by anti-Rh17 antibody, transfusion treatment was required. Therefore, RBC from D-- phenotype has to be available. According to published data, in emergent circumstances when maternal and blood from donor with phenotype D-- is not available, incompatible exchange transfusion is a better choice than delaying transfusion when it is necessary. It is of essential importance that pregnancies with high risk of HDFN due to anti-Rh17 are managed by a multidisciplinary team (transfusion medicine specialist, obstetrician, neonatologist) in a highly specialized tertiary institution.