Proliferation of fibrous tissues to form a localised mass with infiltrating margins may occur anywhere in the body; involvement of the orbit is rare. Children and young adults are most commonly affected. The term 'juvenile fibromatosis' is one of several synonyms. Of the two main forms of this entity, the solitary form is the more common and the prognosis is good. The multicentric form has a poor prognosis. The patients described, a 2 1/2-year-old boy, had a solitary nodule of juvenile fibromatosis removed from the right orbit and was asymptomatic 2 3/4 years postoperatively.