Two cases of congenital syphilis with interstitial keratitis and deafness are reported. The aqueous humor of these patients contained spirochetes which had the characteristic motility and morphology of Treponema pallidum on conventional darkfield examination and which showed affinity for the fluorescein-labeled T pallidum globulin and the characteristic morphology of that organism on conventional darkfield examination. Both patients had received what is generally considered adequate treatment for syphilis several times and had nonreactive serums on standard tests. Even after retreatment with systemic penicillin, the intraocular infection persisted. The importance of the Fluorescent treponemal antibody-absorbtion (FTA-ABS) test in detecting syphilis is stressed, and the value of diagnostic anterior chamber paracentesis is considered in determining whether or not there is active ocular syphilis.