Objective: To highlight the characteristics of pulmonary arterial involvement in Takayasu arteritis. Methods: The clinical and radiological data of a patient with Takayasu arteritis presenting with unilateral pleural effusion were studied and relevant literature was reviewed. The key words, "Takayasu arteritis" and "pleural effusion" were analyzed through literature retrieval in databases. Results: This 58 year-old female patient presented with shortness of breath. The chest CT scan showed bilateral hilar enlargement and pleural effusion on the left side. The blood pressure was not measurable in the course of the disease. After the aorticopulmonary-arteriography, we found that the pulmonary artery and the subclavian artery were involved. The diagnosis of Takayasu arteritis was made, and glucocorticoid therapy was initiated, with significant clinical and radiological improvement after therapy. Literature review found 4 cases of Takayasu arteritis with unilateral pleura effusion, ranging from 32 to 35 years of age, with a female predominance(Female∶Male=3∶1). The chief complaints were fever, chest pain and hemoptysis. All the patients recovered after the treatment of glucocorticoids. Conclusions: Takayasu arteritis presenting with unilateral pleural effusion was easily misdiagnosed as primary pulmonary diseases. Careful physical examination and timely angiography can be used to make the diagnosis.