Background and purpose: To report a case of Coats disease with exudative retinal detachment treated with an intravitreal bevacizumab injection. Methods: Retrospective interventional case report. Results: A 15-year-old boy with early stage 3 Coats disease of the left eye with subretinal exudation and exudative retinal detachment underwent a single injection of intravitreal bevacizumab. A marked reduction in the central retinal thickness (882 to 347 μm) and nearly complete reattachment of retina were noted 3 months after treatment. The best corrected visual acuity improved from counting fingers at 30 cm to 3/60. Conclusions: Intravitreal bevacizumab effectively reduced the subretinal fluid and suggests a vascular endothelial growth factor-mediated component in the pathogenesis of Coats disease in this patient.