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Abstract Pulmonary sequestration with torsion is a rare condition. We describe a seven‐month‐old baby presenting excessive crying for pulmonary sequestration with torsion. Contrast‐enhanced chest computed tomography demonstrated an oval‐shaped mass in the posteromedial right lower chest, no systemic arterial supply was evident. The edge of the mass showed slight linear reinforcement, and its interior had no reinforcement. Thoracoscopic segmentectomy was carried out and histology confirmed pulmonary sequestration with torsion.