AIM: To evaluate the role of intravitreal ranubizumab (IVR) in the treatment of familial exudative vitreoretinopathy (FEVR) of stage 2 or greater either as primary or an ajunct to conventional treatments. METHODS: Retrospective, non-controlled clinical study. Thirty patients (37 eyes) diagnosed with FEVR were enrolled. Twenty patients (66.67%) were male and 10 patients (33.33%) were female. Age ranged from 0.4 to 35 years old (median 3y). IVR was used either as primary or as a combined therapy according to the retinal neovasuclar activities. The follow up ranged from 1 to 57mo with mean 16.73±15.73 (median 11)mo. The treatment effect of retinal neovasuclar activites were recorded as well as the ocular and systemic side effects. RESULTS: Among 30 patients (37 eyes), 10 eyes received single IVR, 1 eye received 2 injections. Three eyes were treated with IVR and simutanous laser photocoagulation. Laser indirect ophthalmoscopy (LIO) was applied in 5 eyes 1mo after the primary IVR. Seven eyes were treated surgically following the primary IVR due to persistent retinal neovasuclar activities and retinal traction. IVR was used as combined treatment with vitrectomy in 11 eyes. Retinal neovascular regression was notified 1mo following the primary IVR in all eyes. Neither systemic nor ocular complications were recorded. CONCLUSION: IVR may be an effective modality in the treatment of FEVR either as primary or as an ajunct to the conventional therapies. The long term effect and safty of IVR still need further research.