Aim: The aim of this study was to investigate the pulmonary magnetic resonance angiography (MRA) imaging findings in Swyer – James syndrome (SJS) as a alternative imaging modalities. Methods: Thirteen patients had posteroanterior inspiratory/expiratory chest X-ray films and pulmonary MRA with SJS (6 males, 7 females) were retrospectively studied. Gradient-echo pulmonary MRA was performed in all patients with a 1.5 T MR unit. MRA imaging was performed in a single breath-hold during the injection of contrast media. Image analysis of the patients was performed. The results of pulmonary artery diameters and pulmonary peripheral vasculature were compared between normal sides versus the affected sides. Statistical analysis was performed with the Student t test. Results: The smaller pulmonary artery on the affected side with poor peripheral vasculature was observed with gadolinium-enhanced gradient-echo MRA in all patients. There was a significant difference between the diameters of the pulmonary arteries of the normal versus the disease sides (p< 0.0005). Conclusion: MRA successfully depicted the small pulmonary artery with diminished peripheral vasculature in patients with SJS. This study showed the pulmonary MRA imaging could be used as a alternative imaging modalities in the evaluation of patient with SJS.