Syphilitic disease of the retina and/or the optic nerve head, without choroidal involvement, occurred in our 4 cases and in another 19 cases. The condition almost always takes place in the secondary stage, frequently associated with meningitis, and rarely in tertiary meningovascular syphilis. Fluctuating visual loss and floating spots without ocular pain are the presenting symptoms. Retinitis, papillitis, and neuroretinitis are accompanied by an inflammatory reaction in the vitreous and, sometimes, in the aqueous. Paracentral scotomas and blind spot enlargement, related with posterior pole and papillary edema, are the most usual visual field defects. Almost complete visual recovery is the rule in the treated cases, although in some instances cystoid macular edema and retinal ischemia due to endarteritis cause permanent visual loss. Treatment with crystalline penicillin is mandatory in patients with concomitant neurosyphilis, whereas procaine penicillin is seemingly sufficient in those with a normal cerebral spinal fluid examination.