Lee et al. (1996) described 2 families that had anomalies of the iris epithelium characterized by cysts and peripupillary ruffles in successive generations. In 1 family, the proband was 3 months old. Both cysts and ruffles were present ... Lee et al. (1996) described 2 families that had anomalies of the iris epithelium characterized by cysts and peripupillary ruffles in successive generations. In 1 family, the proband was 3 months old. Both cysts and ruffles were present in affected individuals, and there was male-to-male transmission. Lee et al. (1996) found 3 previous reports of iris pigment epithelial anomalies in successive generations of a pedigree; the most recent was that reported by Balacco-Gabrieli et al. (1985). Sallo and Hatvani (2002) reported 4 cases of primary pupillary pigment epithelial iris cysts in 3 successive generations of 1 family. Two patients had recurring transitory visual impairment, necessitating treatment. Surgical excision revealed that the cysts were comprised entirely of iris pigment epithelial cells.