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 degree 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. The lobes were untwisted, and lobar fixation was performed to prevent retorsion. Postoperatively, the patient recovered complete lung function, and the patient's chest radiologic and ultrasonographic examination results normalized. Lung torsion is an extremely uncommon event. It causes clinical deterioration in the face of normal arterial blood gases and requires a high index of suspicion for diagnosis. Resection of the twisted lung or lobe may be unnecessary if diagnosis is made early and vascular occlusion is partial. Patients with complete interlobar fissures may benefit from pulmonary lobe fixation as a prophylaxis against occurrence or recurrence of this complication.