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Retinal vein occlusion is a frequent cause of visual loss for which few effective therapies are available. Anticoagulation with low molecular weight heparin might be of value in its treatment. We conducted a systematic review and meta analysis of randomized trials evaluating the effect of low molecular weight heparin in patients with retinal vein occlusion. Data sources included MEDLINE, EMBASE, HealthSTAR, the Cochrane Library, Lilacs, the Investigative Ophthalmology and Visual Science database and gray literature. Main outcome was the mean difference between the visual acuity measured at baseline and at six months expressed in the logMAR scale. Secondary outcome was a composite of any adverse ocular outcome including: worsening of visual acuity, visual fields or fluorescein angiography, or development of iris neovascularization, any neovascularization or neovascular glaucoma. Subgroup analyses for branch versus central retinal vein occlusion were conducted. We identified 1,084 references of which 3 studies comparing low molecular weight heparin with aspirin (229 evaluable patients) were included. Overall, the pooled mean visual acuity difference was −0.23 logMAR (95% CI −0.38, −0.09; P=0.002) in favor of low molecular weight heparin. Low molecular weight heparin was associated with a 78% risk reduction for developing any adverse ocular outcome (pooled RR 0.22; 95% CI 0.10, 0.46; P