Retinal vein occlusion in a woman with heterozygous Fabry's disease

Fabry's disease is associated with high incidence of thrombosis in hemizygo us males and heterozygous females. We describe a woman with Fabry's disease who developed hemi-central retinal vein occlusion during the follow-up. Th e vein occlusion showed a fulminant course ending with a painful blind eye within a short period. Fabry's disease should be considered in the differen tial diagnosis of the vascular occlusive disorders especially in young pati ents.