Severe juvenile arteriosclerosis (JAS) is a rare, fatal disease with probably autosomal recessive inheritance. Precocious arterial murmurs and calcification of aorta and major arteries are associated with anaemia, growth retardation, prolonged systolic hypertension, gradual renal failure, gastrointestinal perforations, and early death. The ophthalmological findings in 5 boys and 1 girl between 7 and 17 years of age were retinal vascular tortuosity of main vessels, pre-capillary arterioles and post-capillary venules, sausage-like bulging of central main arterioles and microaneurysms of arterioles of the central fundus. Narrowing and local constriction was a typical feature of peripheral arterioles. The combination of findings documented by fluorescein angiography in 4 children was typical for severe juvenile arteriosclerosis.