<p>Bronchopulmonary sequestration (BPS) is the second most common congenital cystic lung disease. The size of the lesion generally increases until 25 weeks of gestation and decreases after 28 weeks of gestation. This finding is similar to the reported growth trajectory of congenital pulmonary airway malformation (CPAM), for which the mean CPAM volume ratio (CVR) peaks at 25 weeks of gestation. However, the peak of CVR in patients with extralobar BPSs associated with hydrops is lower than that of the CVR associated with hydrops in patients with CPAM. A CVR cutoff of 0.75 has been utilized as a useful predictor of fetal hydrops and a possible requisite of fetal therapies. We report a case of extralobar pulmonary sequestration. The patient initially showed massive pleural effusion and rightward cardiac deviation at 26 weeks of gestation, followed by the development of hydrops with a CVR of up to 1.5 at 28 weeks of gestation. A total of seven fetal therapies, namely, two maternal steroids, three thoracenteses, and two thoracoamniotic shunts, were performed between 26 and 35 weeks of gestation, resulting in the resolution of hydrops by the 35th week of gestation, shrinkage of BPS at a CVR down to 0.23, and full-term birth by vaginal delivery. Stable perinatal management without any emergent surgical intervention was achieved. Aggressive fetal therapies were very useful for the survival of BPS patients with hydrops and safety in perinatal management.</p>