Four affected family members in two generations demonstrated an unusual, probably autosomal dominant, retinal vascular disorder called inherited retinal venous beading. The proband had retinal neovascularization, exudation, and vitreous hemorrhage. The other three affected family members had significant venous beading and varying degrees of arteriolar tortuosity. Family members with venous beading had low to normal leukocyte and neutrophil counts, which differed significantly from unaffected family members.