Objective The purpose of this study was to show the clinical and imaging characteristics in patients with congenital orbital fibrosis.Methods A retrospective review of a series of 14 patients with congenital orbital fibrosis hospitalized in Institute of Orbital Disease during 2005 to 2009 have been characterized. Patients aged from 3 months to 18 years old,the median age was 7 years old,6 cases (43％) were male.Results Ocular changes were found at birth in all patients,symptoms at onset of the disease included dysfunction of eye movement ( 12 cases ),lagophthalmos (7 cases ),diplopia (5 cases),exophthalmos (4 cases),conjunctival congestion (4 cases ) and endophthalmos ( 2 cases).The results of medical examinations including visual acuity,exophthalmos or endophthalmos,diplopia with or without compensative head station,displacement of the eye, lagophthalmos, blephroptosis and ocular dyskinesia all have been record.In the 14 cases,13 patients have undertaken the CT imaging examination,soft tissue lesions have been found in the orbit with high density and irregular shape,accompanied with thickened ocular muscles and anatomical changes.Of the 9 cases which underwent MRI examination,all of the lesions showed medium signals in T1 WI,with medium to low signals in T2WI.Local excision has been performed in 2 patients,we found that the orbital lesions packaged with hard fibrous tissues around,and the ocular muscles were thick and stark. Pathological examination showed fibrosis and degeneration in the lesions,lacrimal gland and ocular muscles. Some lymphocyte infiltration also has been observed.Conclusions Congenital orbital fibrosis is an orbital disease occurs at birth and is characterized with series of signs such as endophthalmos or exophthalmos,ocular dyskinesia and diplopia.Imaging examinations often find abnormal mass with irregular shape and soft tissue density in orbit.Till now,no efficient therapy has been established.