Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor

Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome is one of the rare forms of congenital fibrosis of extraocular muscles. The nature and cause of such unilateral inflammation leading to fibrosis is unknown. Prenatal orbital penetration has been proposed as a cause of this syndrome. We report a case of this rare syndrome associated with an adjacent sinus tumor.