Retinal arteriovenous malformation (AVM) is a rare unilateral developmental disease that is stable over time in most cases. The authors present a case of a man who was diagnosed with an asymptomatic juxtapapillary AVM of the left eye at the age of 25 years. Visual acuity was 20/20. Fundus pictures realized during an 11-year follow-up disclosed a slowly progressive enlargement of the lesion with vascular loops increasing in number and size. Vision remained stable. No complication was observed. This case demonstrates that retinal AVMs may progressively grow and remain asymptomatic and uncomplicated for a long period of time.