Objective To investigate the reasons, status, treatment and precautions of misdiagnosis of pulmonary inflammatory pseudotumor. Methods Between January 2005 and December 2015, one hundred eighteen articles about pulmonary inflammatory pseudotumor published in Wanfang and CNKI databases were retrospectively analyzed, among them forty-four articles referring to misdiagnosis rate. The misdiagnosis rate, distribute of misdiagnosed diseases, reasons and main means of definite diagnosis were analyzed. Results There were 1 286 cases of pulmonary inflammatory pseudotumor in the 44 articles, of them 1 012 cases were misdiagnosed. The misdiagnosis rate was 78.84%. Pulmonary inflammatory pseudotumor was often misdiagnosed as lung cancer (65.81%), tuberculosis (15.42%, which included 72 cases of tuberculoma and accounted for 7.11%) and benign pulmonary neoplasms (9.59%). Most misdiagnosed patients did not suffer from adverse consequences, except a few patients undergo unnecessary extended operations. Lack of specificity in clinical manifestations, lack of awareness about the disease, dependent on auxiliary examination and lack of awareness about the fine feature of the disease were the main reasons of misdiagnosis. The majority of misdiagnosed cases were terminal pathological diagnosed through the operation or after percutaneous biopsy. Conclusions Pulmonary inflammatory pseudotumor is lack of specificity in clinical manifestations and easy to be misdiagnosed. It is very important to analyze and identify the fine feature of imaging changes. To reduce and avoid misdiagnosis, clinicians should improve the awareness of this disease.