BACKGROUND: Placental chorioangioma is a benign tumour of the placenta. In most cases, chorioangiomas are small and do not result in maternal or fetal complications. This report presents a case with a large placental chorioangioma, which was associated with an intrapartum event leading to significant maternal and perinatal morbidity. CASE PRESENTATION: A case of placental chorioangioma was diagnosed at 29 + 1 weeks of gestation. As the tumour enlarged progressively with polyhydramnios, further ultrasound examination indicated signs of fetal hydrops. Following multi-disciplinary team discussion, caesarean section was performed with delivery of a female infant. Pathological examination of the placenta confirmed an infarcted chorioangioma. CONCLUSIONS: Placental chorioangiomas are benign, non-trophoblastic vascular neoplasms. While often asymptomatic, they can occasionally have unfavourable outcomes. Ultrasound examination plays a critical role in their diagnosis and monitoring. These neoplasms exhibit a wide range of clinical manifestations, leading to their classification as a spectrum of symptoms rather than a distinct syndrome. Early diagnosis, vigilant prenatal surveillance, and timely intervention are essential to minimize fetal morbidity and mortality.