Pulmonary arteries involvement is well described in Takayasu's arteritis (TA), a condition which is mainly associated with involvement of the systemic arteries. We report a case of TA with documented isolated pulmonary arteries involvement. Symptoms were quite similar to those encountered in chronic thromboembolic disease. A pulmonary angiogram showed bilateral stenosis and occlusion of pulmonary arteries. Diagnosis of TA was suspected, and as such a complete aortogram was made but proved to be normal. Massive haemoptysis suddenly occurred, which resulted in death. Autopsy disclosed characteristic pathological lesions of TA in pulmonary arteries and confirmed the lack of involvement of the aorta and its branches. The frequency of such a clinical form could be underestimated, given the difficulties of diagnosis and features similar to those of chronic thromboembolic disease.