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To describe a case of incontinentia pigmenti with proliferative retinopathy successfully treated by combination of repeated retinal laser photocoagulation and intravitreal ranibizumab injection.Single interventional case report of a 4-year-old girl, known case of incontientia pigmenti, first screened at the age of two, presented with proliferative retinopathy. Sole treatment by panretinal laser photocoagulation failed to control the progression of retinal neovascularization. Intravitreal ranibizumab injections were used as an adjunct therapy.The proliferative retinopathy was brought under control and halted till this date with repeated intravitreal ranibizumab and panretinal photocoagulation. No systemic side effect was noted.Intravitreal ranibizumab can be considered as an adjunct therapy in proliferative retinopathy secondary to incontinentia pigmenti when monotherapy with panretinal photocoagulation fails. Intravitreal ranibizumab may quickly inhibit the intraocular vascular endothelial growth factor and may prevent devastating complications like tractional retinal detachment. Extra caution has to be taken in adopting different dosages and injection regimes especially in pediatric cases.