We report herein the cases of two infants who developed right pneumone
ctomy syndrome, both of whom were born with gross C-type esophageal at
resia (EA/TEF), and a hypoplastic right lung arising from the lower es
ophagus, being a bronchopulmonary foregut malformation (BPFM). Appropr
iate and well-timed treatments for a variety of sequelae primarily cau
sed by the mediastinal shift must be considered after right pneumonect
omy in early childhood.