Antiphospholipid syndrome (APS) is a hypercoagulable disorder with highly variable symptomatology including ocular manifestations. Antiphospholipid antibodies (aPL) are the serologic markers of this clinical entity. Our aim was to note the main associated ocular features of APS and elucidate which pathogenic mechanisms may participate. Retinal vascular thrombosis and different neuro-ophthalmologic manifestations, such as optic neuropathy and amaurosis fugax, may be considered as the ocular hallmarks of this syndrome. Ocular features due to aPL-induced thrombosis should be treated with anticoagulant drugs. Conversely, for the treatment of ocular features due to immunological mechanisms such as vasculitis, immunosuppressive regimes seem to be more appropriate.