Sarcoma of the pulmonary artery is a rare tumor. The histopathologic f
eatures of these tumors differ widely, with leiomyosarcomas representi
ng less than 20 % of all pulmonary sarcomas. Diagnosis is very difficu
lt because most of the cases have no specific clinical signs: the majo
r differential diagnosis is that of pulmonary thromboembolism. We repo
rt the case of a 45-year-old woman, who was first treated for pulmonar
y thromboembolism until the diagnosis of an intravascular growing tumo
r was made by means of computed tomography, pulmonary angiography and
nuclear magnetic resonance tomography. The patient underwent right pne
umonectomy and microscopically a leiomyosarcoma of the right pulmonary
artery was found.