A 32-year-old active cyclist was referred for the evaluation because of syncope he had performed in preseason. He was found to be normostenic, acyanotic, normotensive with clear lungs and a regular pulse of 60 bpm, with normal dual heart sounds and a grade Levine 2/6 continuous diastolic murmur in the second intercostal space of the left parasternal area. He had no family history of premature cardiac death and his lipids were normal. Rest ECG showed a regular sinus rhythm of 62 bpm with incomplete right bundle branch block and no significant ST-T changes. A transthoracic ECHO in parasternal short-axis view revealed an anomalous colour flow jet in diastole arising from the lateral wall into the main pulmonary artery and coronary artery fistula with non-significant left-to-right shunt (Qp/Qs ratio 1.2).came under suspicion. In contrast, it has not been confirmed clearly by the transoesophageal ECHO. Coronary angiography was without coronary stenosis and confirmed a 'serpentine' anomalous drainage supposedly from left anterior descending artery to the main pulmonary artery. A complex anatomy of sacculary dilated fistula that originates from the proximal left anterior descending artery and drainages the main pulmonary artery was showed in detail by a 64 slice MDCT scanning. Myocardial Tc-99m Myoview perfusion SPECT imaging showed no perfusion defects in maximal physical stress and follow-up without intervention was suggested. In addition, due to the results of holter ECG monitoring, head-up tilt testing, carotid sinus massage and programmed atrial stimulation syncope was concluded as vasovagal, but its nature still remains discussible. After a three year follow-up patient remains asymptomatic and recently performed control myocardial perfusion SPECT showed no signs of stress related myocardial ischemia. In conclusion, several imaging techniques are needed for an accurate diagnosis of coronary fistula and for the suggestion of proper further management. In some cases syncope may be the first manifestation of CAF, but it is still unclear if it is directly related to the coronary anomaly.