Objective To analyze the imaging manifestation and feature of congenial pulmonary arteriovenous fistulas (CPAVF) and observe the effect of endovascular treatment. Methods The plain films, CT and DSA of CPAVF retrospective- ly were analyzed. The effect of transcatheter embolization with coil was observed by follow-up. Results Feeding artery was pulmonary artery branches in five cases and was branchial artery in one case. The images of all cases were divided into three types: simple type-3 cases, complex type-2 cases and diffuse type-1 case. Both CT and DSA could give the correct diagnosis, however 3 cases were diagnosed wrongly by X-ray. Three cases were underwent transcatheter endovascular em- bolism with coils, and one case were done three times. Systemic arterial oxygen saturation improved strikingly in two pa- tients after treatment, and the symptoms of hemoptsis disappeared immediately after branchial arterial embolism in other case. Conclusion DSA was the best means in the diagnosis of PAVF. CT and three-dimensional reconstruction can be giv- en correct and direct diagnosis for big PAVF, but can not replace DSA in the diagnosis of small PAVF. Percutaneous tran- scatheter endovascular embolism with coils is an effective and safe treatment for PAVF, but it is only a palliative therapy for diffuse PAVF.