Objective: The goal of this study was to summarize the clinical data from patients with coronary artery fistulae (CAFs), to identify clinical features of CAFs suggestive of this diagnosis, and to illustrate the 64-Slice Computed Tomography (CT) findings of various types of CAFs in adults. Methods: We retrospectively reviewed the medical records of 11 patients with CAFs confirmed by coronary angiography or surgery, and carefully analyzed their clinical data. We also present the 64-slice CT images of these CAFs, which are critical in determining optimal management. Results: Two (18%) of 11 patients were <20 years of age at the onset of clinical symptoms. Both are presented with recurrent pneumonia. There were two patients >20 but <30 years of age, both of whom experienced recurrent upper respiratory infections or pneumonia and tachynea/dyspnea (one patient), or chest tightness/angina (one patient). All patients >30 years of age (67%) presented with chest tightness (57%), tachypnea/dyspnea (42%), palpitations and dizziness (28%), without recurrent upper respiratory infections or pneumonia. No patient <30 years of age had fistula-related complications, but all older patients (>30 years of age) had complications. As to Cardiac murmur and the ECG, there were not any definite rules. Ten patients (91%) were correctly diagnosed by 64-slice CT. Conclusions: When careful consideration of the clinical manifestations, physical examination, and the age of the patient brings CAFs into the different diagnosis, the 64-slice CT can help us to confirm and establish the diagnosis. Thus, patients can obtain optimal treatment in order to decrease the morbidity and mortality associated with CAFs.