The clinical course of primary fetal hydrothorax is unpredictable. Whereas smaller unilateral effusions might remain stable or even regress, this is rarely the case with larger collections. Bilateral effusions, hydrops, preterm delivery and the lack of antenatal therapy are all associated with poor outcome. Once structural and chromosomal anomalies have been excluded, optimal management depends on gestational age, rate of progression, the development of hydrops and associated maternal symptoms. For very large effusions with mediastinal shift, hydrops and/or hydramnios, or when there is rapid enlargement of the effusion, fetal intervention is warranted. Survival can be maximized by pleuroamniotic shunting, which can reverse hydrops and hydramnios and prevent pulmonary hypoplasia. Pleuroamniotic shunting can also be used for the treatment of other large cystic lung lesions, such as a macrocystic congenital cystic adenomatoid malformation or bronchopulmonary sequestration, especially when associated with hydrops.