A 12-year-old patient presents aniridia, congenital cataract and nystagmus. The zonular cataract at both eyes has evolved at the right eye. Visual acuity at the right eye is very low (hand movement perception). The lens intracapsular removal at the right eye, with the resection of the hyaloid-capsular ligament, has improved the visual function at 1/6 with adequate correction. The specificity of the case resides in his infrequent occurrence, probably because of a genetic mutation causing partial lack of the zonular fibers.