Introduction: Primary pulmonary angiosarcoma (PPA) is a very rare tumour with a grave and short prognosis and whose primary site is difficult to confirm. The diagnosis is essentially histological and immunohistochemical because there are no specific clinical or radiological signs.
Observation: We report a case of a man with PPA, discovered during the investigation of chest pain. Imaging (x-ray and CT) identified a right apical mass. The diagnosis was made by histological and immunohistochemical analysis of CT guided percutaneous biopsies.
Conclusion: As a result of this observation we propose further studies on this extremely rare and grave condition.