Congenital unilateral absence of orbit with anophthalmos and partial arhinia.

Congenital unilateral absence of orbit is a very rare clinical entity. An 18 month old female child with an unilateral absence of orbit with clinical anophthalmos and partial arhinia is presented. X-ray of the skull revealed an absence of the bony orbit on the left side. Unlike previously described cases of absence of the orbit, this maldevelopment is unilateral and present in an otherwise normal thriving child.