We report the clinicohistopathological findings of the proliferative vitreoretinopathy in an eye with Coats' disease using the tissue obtained during surgery. A 28-year-old man, who had experienced poor vision in his right eye for a period of 4 years, was referred to our hospital. Examination revealed an extensive yellow exudate in the subretinal space and a tractional retinal detachment. Prominent teleangiectatic retinal vessels were also temporally present. Evident epiretinal membranes were present in the postequatorial area. We performed a scleral buckling, vitrectomy, membrane peeling, endophotocoagulation and silicone oil tamponade. A histological examination revealed that the epiretinal membrane consisted of collagen fibers, glial proliferation, foam cells and lymphocytes. The foam cell in the epiretinal membrane is a characteristic finding in an eye with Coats' disease.