RATIONALE: Coronary artery perforation (CAP) is one of the most serious complications of percutaneous coronary intervention. Coronary arteriovenous fistula is a considerably rare type of congenital CAP. They are usually difficult to distinguish. PATIENT CONCERNS: A male patient developed coronary artery perforation during percutaneous coronary intervention. As balloon occlusion was ineffective, a decision was made to implant a spring coil and bypass the occluded segment. However, the placement of spring coils restored patency in the occluded distal segment of the right coronary artery (RCA). DIAGNOSES: Coronary computed tomography angiography is an auxiliary tool and Digital Subtraction Angiography(DSA) is the gold standard for the diagnosis. INTERVENTIONS: Surgery and implant a spring coil are the main treatment methods. OUTCOMES: The patient's RCA regained its blood supply. Coronary artery recanalization arteries occurred after spring coil placement. Combined with the imaging presentation, we finally determined that he was coronary-right ventricular fistula. LESSONS: Although congenital CAPs are rare, appropriate detection and timely confirmation by coronary angiography are important for determining their subsequent management. Congenital coronary arteriovenous fistulae may be considered when coronary artery perforation during percutaneous coronary intervention, balloon blockade is ineffective and the patient's vital signs are stable.