Congenital pulmonary airway malformation (CPAM) is a hamartoma-like lesion characterized by a polycystic mass of lung tissue with abnormal bronchial proliferation. It is the most frequent congenital thoracic malformation, accounting for 30–47% of fetal thoracic lung lesions. The exact pathogenesis is still unknown. Comorbidities associated with CPAM include pulmonary sequestration, lung malignancy, polyhydramnios, fetal hydrops, agenesis of the corpus callosum, congenital nephrotic syndrome, cleft lip and cleft palate, renal agenesis, esophageal atresia, diaphragmatic hernia, cardiovascular, and skeletal defects. Histopathological classification consists of five types of lesions; however, the most common clinical practice is to describe the CPAM lesion as microcystic (<5 mm) or macrocystic (>5 mm) and state its size. Microcystic lesions are associated with fetal hydrops and have a poor prognosis, whereas macrocystic lesions are usually not associated with hydrops and have a favorable prognosis. Ultrasound (US) scans every 2 weeks are recommended to monitor growth, lung lesions, and amniotic fluid volume for possible complications. Around 40% of CPAMs increase with the gestational age and stabilize around 26 weeks of gestation; however, in some cases, these lesions may continue to grow late into the third trimester. Fetal hydrops occur in about 5% and are associated with a close to 100% mortality rate. In cases of fetal hydrops, prenatal interventions may allow survival to birth. In the absence of contraindications, term delivery is recommended at a tertiary care center. In cases of poor growth, fetal hypoxia, or hydrops, earlier delivery is recommended for immediate postpartum treatment. About 70% of the children are asymptomatic at birth. For symptomatic infants, surgical resection is the management of choice.