BACKGROUND: Posterior polymorphous dystrophy has been described in the scientific literature as having more than a casual association with keratoconus in adults. We studied four consecutive children with posterior polymorphous dystrophy who had coexisting astigmatism. In our patients, the posterior changes appeared to be associated with the corneal astigmatism, as evidenced by videokeratography. METHODS: Four consecutive cases of posterior polymorphous dystrophy in children were found over a 3-year period by one of the authors (PD). They presented with varying degrees of amblyopia in the eyes with the greater amount of posterior polymorphous dystrophy. Cycloplegic refractions and keratometry readings were performed . The more affected eyes had greater degrees of astigmatism, presumably causing the amblyopia. Whenever possible, the results were supplemented by confirmation by a corneal specialist, specular microscopy, slit-lamp photographs and videokeratography. RESULTS: Greater asymmetry of posterior polymorphous dystrophy was associated with greater astigmatism in the more affected eye. When more astigmatic anisometropia was present there was a greater amblyopia. Videokeratography of one of the patients showed that an area of confluent blebs forming a crescent shape compared exactly with a steep cylinder along the same axis. With-the-rule astigmatism was associated with a more diffuse pattern of blebs. CONCLUSION: Decreases in visual acuity in children with posterior polymorphous dystrophy may sometimes be due to an amblyopia caused by the astigmatism of asymmetric disease. [J Refract Surg. 1996;12:709-714.]