Primary retinal telangiectasis or Coats' disease is a non-hereditary r
etinal vascular abnormality consisting of incompetent telangiectatic a
nd aneurysmal retinal vessels, It is characteristically found unilater
ally in boys and occasionally may be associated with other systemic di
sorders. The authors report the first case of primary retinal telangie
ctasis with a concomitant diffuse central nervous system venous abnorm
ality.