Retinal vein occlusion (RVO) is the second most common retinal vascular disease after diabetic retinopathy and is a common cause of visual morbidity and blindness in the elderly. A large proportion of patients with RVO have a history of cardiovascular disease, hypertension, diabetes mellitus or open-angle glaucoma. Although RVO is sometimes associated with thrombophilias and coagulation abnormalities, the role of coagulation factors in the development of RVO remains unclear. This review did not find strong evidence to support an extensive work-up for thrombophilic and coagulation diseases for the vast majority of patients. However, when tests for common cardiovascular risk factors for RVO are negative, evaluation for potential coagulation disorders may be indicated, particularly in young patients and in patients with bilateral RVO, a history of previous thromboses or a family history of thrombosis.