The multiple organ system involvement of congenital generalized fibromatosis is usually severe enough to result in the demise of the infant within the first few months of life. The present case is an exception. It represents what appears to be the first report of a primary fibrous tumor of the cornea and the first report of ocular involvement in this uncommon syndrome. The infiltrative nature of fibromatosis makes complete excision difficult and recurrences common. A cure could not be obtained by either radiation or excision.