Incontinentia pigmenti (IP) is an X-linked dominant disorder affecting the skin, teeth, eyes, and central nervous system. Ocular changes are common and may lead to severe vision loss. We report on the ocular manifestations in two young girls with IP, with emphasis on the asymmetry of this condition in both eyes and associated retinal problems. The outcomes of laser treatment of the ischemic peripheral retina were good and resulted in stability of vision.