Pierre Robin sequence (PRS) is a complex congenital condition characterized by a small lower jaw (micrognathia), a cleft palate, and a tongue positioned backward in the mouth (glossoptosis). It can be isolated but is often associated with other genetic syndromes. Pierre Robin sequence is associated with a number of ocular complications including myopia, strabismus, Möbius syndrome, nasolacrimal duct obstruction, glaucoma, cataracts, microphthalmia, choroid coloboma, and retinal detachment. Collaboration with other specialists for comprehensive care is important.