We determined the clinical characteristics of cataract in 133 patients with the Stickler syndrome. Cataracts of various types or aphakia were found in 115 of 231 eyes (49.8%) studied. The most frequent and distinctive lesions, described as wedge and fleck cataracts, accounted for 40 of the 93 cataracts (43.0%) observed. These distinctive opacities may serve as a clinical marker for the Stickler syndrome and facilitate early diagnosis.