Objective To summarize the clinical characteristics of polyarteritis nodosa which begin with pulmonary lesions, so as to make early diagnosis and treatment possible. Methods Clinical data of three patients of polyarteritis nodosawhich began with pulmonary lesions were summarized includingmode of onset, evolvement of symptom and sign, data of laboratory test. The results of vascular ultrasound and histopathology examination were analyzed for their diagnostic value.Results Cough, sputum productive cough, and irregular high fever were present in the earlier period. Increases of C-reactive protein ( CRP) , erythrocyte sedimentation rate ( ERS) , white blood cell count ( WBC) , and anemia were main laboratory findings. Computed tomography revealed scattered infiltration in the lung. Anti-infective treatment was ineffective. Involvement of skin, kidney, gastrointestinal tract, nerve and muscle was present in sequence. Two of the three cases were confirmed by pathological biopsy. The symptoms were improved by the treatment with glucocorticoid. Conclusions Polyarteritis nodosa which begin with pulmonary lesions is easy to misdiagnose due to atypical symptoms. It is important for diagnosis of polyarteritis nodosa to collect evidence of systematic involvement through taking careful history and physical examination. Further angiography and biopsy can confirm the diagnosis. Cytotoxic drugs and/ or glucocorticoid are effective for the treatment of polyarteritis nodosa.