Posterior lenticonus is associated with progressive lens opacification. We report a series of 40 consecutive patients (41 eyes) with posterior lenticonus who were treated by three pediatric ophthalmologists between 1974 and 1988. Prior to cataract surgery, appropriate patients were treated with atropine dilatation, spectacle correction including bifocals for refractive errors, and amblyopia occlusion therapy. Indications for surgery were a measured decrease in visual acuity, the loss of a central fixation reflex, or the onset of strabismus. The age at which cataract surgery was performed ranged from 2 months to 12 years (mean 4 years, 6 months). Nineteen eyes (49%) achieved postoperative acuities in the 20/20 to 20/40 range, 7(18%) eyes achieved 20/50 to 20/100, 4 (10%) eyes achieved 20/200, and 4 (10%) eyes achieved less than 20/200. Two (5%) young patients had central, steady, and maintained visual fixation reflexes and 3 (8%) additional patients had central, steady, but not maintained reflexes. After cataract surgery and aphakic optical correction, amblyopia was present in 84% of patients and strabismus was present in 51% of patients. The patients in this study had sufficient optical distortion to produce amblyopia. Cataract removal and optical correction alone did not correct the vision; occlusion therapy for amblyopia was required. Earlier surgery may be indicated to prevent visual deprivation amblyopia in patients with posterior lenticonus.