Retinal vein occlusion (RVO) is the second most common cause of visual impairment due to retinal disease after diabetic retinopathy. Nowadays, the introduction of new, powerful diagnostic tools, such as spectral domain optical coherence tomography, and the widespread diffusion of intravitreal drugs, such as vascular endothelial grow factor inhibitors or implantable steroids, have dramatically changed the management and prognosis of RVO. The authors aim to summarize and review the main clinical, diagnostic, and therapeutic aspects of this condition. The authors conducted a review of the most relevant clinical trials and observational studies published within the last 30 years using a keyword search of MEDLINE, EMBASE, Current Contents, and Cochrane Library. Furthermore, for all treatments discussed, the level of evidence supporting its use, as per the US Preventive Task Force Ranking System, is provided.