Familial exudative vitreoretinopathy complicated with macular hole retinal detachment

Familial exudative vitreoretinopathy (FEVR) is a rare genetically heterogeneous inherited disorder with varied presentation. We report a case of FEVR with a classic tractional component and a rare co-occurrence of macular hole with subtotal retinal detachment. The diagnosis was made on clinical examination and imaging. There have been four cases of full-thickness macular holes reported in the literature and none with macular hole retinal detachment. The patient underwent vitrectomy for the same and had silicone oil removal with attached retina postoperatively. We want to highlight this rare association and emphasize the importance of regular follow-up of FEVR cases which tend to progress after remaining quiescent for years.