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Idiopathic Pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease with unknown cause, which is closely related to lung cancer. A serious complication called Acute exacerbation of IPF (AE-IPF) is prone to occur after lung resection. It progresses rapidly without effective treatment and has a poor prognosis. A typical case of AE-IPF after lung cancer surgery was reported, and its clinical characteristics, imaging features, diagnosis and treatment were summarized.