Empty sella syndrome (ESS) is a peculiar disease that may involve systemic, neurological or ophthalmological symptoms. Our report presents a case of primary ESS complicated with central retinal vein occlusion. In an attempt to explain the pathogenesis of the retinal venous stasis, it may be hypothesized that the compression of the optic nerve may produce a slowing of axoplasmic flow, with a consequent optic nerve head swelling. Thus the central retinal vein gets compressed with the clinical result of a retinal venous stasis.