A 6-year-old girl presented with left divergent strabismus that had been noticed by her parents. She was known to have Turner's syndrome. On examination, she had tractional retinal detachment in the left eye and a temporal avascular area with neovascularization at the junction with the vascular area. Clinical features were characteristic of familial exudative vitreoretinopathy. This case is a rare and, to the authors' knowledge, the first reported association of familial exudative vitreoretinopathy and Turner's syndrome.