IgG4-related peripulmonary arteritis is rare. A 60-year-old woman presented with fatigue for more than 2 years. Transthoracic echocardiography revealed a lesion measuring 2.1 × 1.5 cm in the pulmonary artery bifurcation. CT pulmonary angiography showed marked stenosis of the right and left pulmonary arteries. FDG PET/CT showed increased FDG uptake of the thickened walls in the bilateral pulmonary arteries with SUVmax of 5.8. The patient underwent partial resection of the pulmonary artery lesion. IgG4-related peripulmonary arteritis was confirmed by pathology.