A 13-year-old boy was referred because of visual deterioration in his right eye. The visual acuity was two meters of counting fingers. Indirect ophthalmoscopy and biomicroscopy revealed exudative macular edema as well as tumor-like telangiectatic vessels and exudation in temporal periphery. With diagnosis of Coats' disease (stage II) confirmed by fluorescein angiography, three intravitreal injections of bevacizumab were performed at 6-week intervals. One year after the last injection, there was a significant resolution of macular edema as well as visual acuity improvement to 20/20. This is the first case report in which a distinct improvement in macular edema was observed with intravitreal bevacizumab in Coats' disease.