Mondino and Brown (1976) described a family of Irish descent in which 5 persons in 4 generations showed markedly deficient lacrimation from infancy and punctate corneal epithelial erosions. Male-to-male transmission was observed. Hypoplasia of the lacrimal glands was ... Mondino and Brown (1976) described a family of Irish descent in which 5 persons in 4 generations showed markedly deficient lacrimation from infancy and punctate corneal epithelial erosions. Male-to-male transmission was observed. Hypoplasia of the lacrimal glands was suggested by pharmacologic tests and histopathology of the lacrimal gland. There were no associated ocular or adnexal abnormalities. Alacrima occurs in anhidrotic ectodermal dysplasia (305100) and dysautonomia (223900) and in association with ocular and adnexal abnormalities. Krueger (1954) described brother and sister with ptosis, distichiasis, conjunctivitis, keratitis, and alacrimia congenita. The father and another brother were said to have defective lacrimation. A nuclear defect was postulated for this disorder, which may be distinct from that reported by Mondino and Brown (1976).