Although bilateral Coats disease associated with severe FSHD has been reported in 3 girls,2,3 this has not previously been reported in the literature in an asymptomatic adult patient to our knowledge. Other diagnoses that can manifest similarly were also considered in this patient prior to genetic testing. They include vasoproliferative tumor, familial exudative vitreoretinopathy, dyskeratosis congenita, and Parry-Romberg syndrome. However, there were no feeder vessels (as are sometimes seen in vasoproliferative tumor), no family history of retinal disease (seen with familial exudative vitreoretinopathy), no bone marrow, skin, or nail abnormalities (found in dyskeratosis congenita), and no hemifacial atrophy (as in Parry-Romberg syndrome). Despite the unique manifestation, the clinical picture in conjunction with genetic testing suggested a Coats-like disease in association with FSHD. Genetic testing was critical as the patient did not initially show any symptoms of muscular dystrophy and did not manifest any electromyographic abnormalities.