To clarify the value of clinical long-term follow-up with radiological examination, ranging from 12 to 63 months (average: 35 months), 18 consecutive patients suffering from spontaneous carotid cavernous fistula (CCF), were studied prospectively. Five aged patients without aggressive symptoms were treated conservatively, and the other 13 underwent transarterial embolization. The radiological follow-up was primarily by magnetic resonance angiography (MRA), performed from 2 to 6 times (average: 4.1 times) during the follow-up period. In three cases, CCFs persisted, but the other fifteen (83%) demonstrated complete cure as defined by long-term follow-up MRA. The three patients with persistent CCFs were comparatively young, less than 60 years old, had no atherosclerotic factors and demonstrated multiple venous drainage routes with cortical venous drainage on angiography. In two of them, the symptoms completely disappeared, and the other had only mild chemosis. However, surprisingly, in two, MRA revealed residual CCF with drainage into only cortical veins through the sphenoparietal sinus, this radiological finding being well known to signify danger. During the follow-up period, central retinal vein thrombosis occurred in two cases. The common point in these cases was that the superior ophthalmic vein was the only venous drainage route. This is also a point requiring care. We therefore emphasize the importance of careful long-term radiological follow-up for spontaneous CCF patients even when their symptoms improve or disappear. MRA is particularly suitable for this purpose and applicable in the out-patient clinic because of its non-invasive nature.