Background: To assess the prevalence of thrombophilia in patients with central (CRVO) and branch retinal vein occlusion (BRVO). Methods: In 139 patients with CRVO (n = 88) and BRVO (n = 51) and in 40 healthy controls factor VIII, fibrinogen, antithrombin III, protein C, protein S, activated protein C resistance, anticardiolipin antibodies (ACA), homocysteine, factor V Leiden, prothrombin G20210A and methylene tetrahydrofolate reductase (MTHFR) C677T mutation were assessed retrospectively. Results: Elevated factor VIII activity and the homozygous MTHFR C677T mutation were significantly more often found in CRVO and BRVO cases compared to controls. Age-, gender- and C-reactive protein-adjusted logistic regression analysis did not show a significant additive effect of elevated factor VIII activity on the risk of developing CRVO/BRVO. Elevated fibrinogen levels and ACA were significantly more often found in CRVO than amongst controls. No significant differences were found concerning the remaining variables. Conclusions: We suggest elevated fibrinogen levels, ACA and the homozygous MTHFR C677T mutation as potential risk factors for CRVO/BRVO.