Ballantyne syndrome is a rare obstetric condition characterized by fetal hydrops, maternal edema, and placentomegaly. Its low incidence, combined with potential clinical overlap with preeclampsia, poses significant diagnostic and management challenges. This condition is especially relevant due to the severe complications it can cause during pregnancy. In this article, we present a case of Ballantyne syndrome associated with Down syndrome (DS) and chorioamnionitis, complemented by a literature review exploring its possible etiologies, pathophysiology, and therapeutic options. Additionally, the importance of early diagnosis, the use of invasive techniques to identify underlying etiologies, and therapeutic decision-making focused on safeguarding maternal life are emphasized, including pregnancy termination in cases of severe complications to optimize maternal and fetal outcomes.