Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life-threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in adults and children. From the literature between 1993 and 2004, 236 patients with CAFs were considered for evaluation. The present review is intended to assist cardiologists who are unfamiliar with congenital CAFs in adults by suggesting clues for decision making regarding diagnosis and management. Dyspnea and chest pain represented a frequent 91/128 (71%) clinical symptom in CAFs in adults while in the pediatric age group the majority were silent 105/133 (79%) and dyspnea and chest pain accounted for only 8% of the symptoms. The diagnostic modalities were mainly cardiac catheterization and coronary angiography. On the other hand, in the pediatric patients, echocardiography and coronary angiography mainly guided the diagnosis. Regarding treatment strategy in the reviewed subjects, percutaneous transluminal embolization was performed in 18% of the pediatric and in only 5% of the adult subjects. Surgical ligation (46% vs. 38%) and conservative medical strategies (36% vs. 24%) were reported in both pediatric and adult groups. Presentations of CAFs vary considerably in both groups. These differences include the diagnostic modalities, spontaneous closure, spontaneous rupture, and management. From this review, it seemed that--but it may be biased--surgical ligation remains the major mainstay for closure of CAFs in adult and pediatric populations. Recommendations are necessary for antibiotic prophylaxis and antiplatelet and/or anticoagulant therapy for prevention of endocarditis and thrombotic events in patients with CAFs associated with coronary artery dilatation or aneurysmal formation of the fistulous tract.