Lung torsion is a very rare event that has been described after trauma, spontaneously, and post–thoracic surgery, with only 8 cases reported in the pediatric literature. We present the first case report of lung torsion complicating tracheoesophageal fistula repair. The diagnosis was suggested on chest ultrasonogram and Doppler and confirmed by computed tomographic scans. On exploration, a 90° rotation of the right middle and lower lobes in a clockwise direction was found. A complete interlobar fissure and an absent inferior pulmonary ligament were identified as predisposing factors.