Background: Pulmonary sequestration (PS) associated with massive hemoptysis, hemothorax, and elevated tumor markers or even diagnosed with lung malignancy has been reported many times. But all these clinical features have never been reported in the patient diagnosed with PS without malignant disease.
Case presentation: A 45-year-old man with PS presented with massive hemoptysis, hemothorax, extremely elevated carcinoembryonic antigen (CEA) in the pleural effusion, was initially misdiagnosed with advanced lung carcinoma, and was finally diagnosed with PS with Aspergillus infection.
Conclusions: PS is rarely concurrent with lung cancer; most of the time, it is easily misdiagnosed as a malignancy, especially when presenting with a fungal infection, which could remarkably elevate CEA in the pleural effusion.