Congenital coronary artery fistulas (CAFs) constitute an unusual cardiovascular anomaly. The aneurysmal appearance of CAFs is not uncommon and depends on the shunt size. However, few cases of ectatic coronary arteries (type III according to Markis et al. classification-diffuse ectasia in one vessel) supplying the fistulas have been reported. Below, we report the case of a 65-year-old woman, who referred to our department because of worsening exertional dyspnea. Echocardiographic evaluation, both transthoracic and transesophageal, performed after admission disclosed a giant tortuous ectatic right coronary artery with a fistulous connection to the coronary sinus. The presence of the CAF was confirmed by cardiac catheterization. In addition, we discuss the pathophysiology of the above congenital anomaly, as well as its management.