Coronary artery fistula (CAF) is a rare condition, whilst lung cancer is one of the most common malignant tumors worldwide. We came cross an interesting case with both diseases. To the best of our knowledge, this is the first case report pertaining to a patient with a coexisting CAF and lung adenocarcinoma. The patient was a 67-year-old woman who was admitted to our hospital for evaluation of persistent cough. Through the examination she was diagnosed coronary artery fistula and lung adenocarcinoma. Both diseases were successfully treated in a single operation (artery ligation and pulmonary lobectomy). The post-operative period was uneventful. At 3-month follow-up, there were no signs of blood shunting or cancer recurrence. There is no standard guidelines to treat both diseases. We want to seek out a solution to the problem. In this patient, we successfully performed artery ligation and pulmonary lobectomy in a single operation without any complications. We believe the treatment of patients with CAFs should be individualized. But, there is still a lot of shortcomings in our research. First of all, we have no enough cases to support our approach. What's more, the long-term effects of the operation are not certain. Last but not least, we have no proof in genetics with both diseases.