Abstract Background Woven coronary artery (WCA) is an extremely rare congenital anomaly in which a part of epicardial coronary artery is divided into thin channels, that twist along the axis of the coronary arteries and then merge again as the main coronary lumen. This anomaly is regarded as a benign condition because the blood flow is normal. Very few cases of WCA have been reported. Case presentation Herein we report a case of a 44-year-old man who was admitted to our hospital due to 20 years of repeated episodes of heart palpitations, 2 years of shortness of breath after activity, and the symptoms were aggravated for 1 month. He had history of inferior myocardial infarction and atrial fibrillation. Color Doppler echocardiography revealed rheumatic heart disease, severe mitral regurgitation, mild-moderate tricuspid regurgitation, moderate pulmonary hypertension. Coronary angiography revealed 60–85% diffuse stenosis in the middle of left anterior descending artery, 60–90% diffuse stenosis in the middle of left circumflex artery, 30–40% diffuse stenosis in the proximal segment of right coronary artery, and WCA anomaly in the middle, and distal segments of right coronary artery. Conclusion The patient successfully underwent prosthetic valve replacement and left anterior descending coronary artery bypass grafting, and had a good recovery after surgery. Further studies are needed to fully understand the disease and determine appropriate treatment options.