A case is reported of a five-month-old female with incontinentia pigmenti associated with nasolacrimal duct obstruction. Ocular manifestations of incontinentia pigmenti have previously been described to include persistent hyperplastic primary vitreous, retinal dysplasia, retrolental fibroplasia, corneal opacities, cataract, optic atrophy, and strabismus. This case is believed to be the first reported instance of incontinentia pigmenti associated with nasolacrimal duct obstruction. The management of this patient is also discussed.