Carotid cavernous fistulas are pathologic connections between the internal and/or external carotid artery and the cavernous sinus. According to Barrow one can distinguish between direct (high flow) and indirect (low flow) fistulas, whereby direct fistulas are often traumatic while indirect fistulas more frequently occur spontaneously in postmenopausal women. Diagnosis can easily be established using MRI and angiography, which allow exact visualization of the anatomy of fistulas to plan the interventional neurological therapy that in recent years has replaced surgical therapy. This article provides an overview on imaging findings, diagnosis using MRI and angiography as well as interventional treatment strategies.