Purpose: To describe an option of surgically draining large macular cystoid spaces in a patient with Coats disease. Methods: A case and its findings were analyzed. Results: A standard pars plana vitrectomy was performed to aspirate large macular cystoid spaces using a subretinal cannula with intraoperative optical coherence tomography guidance. Because of the viscous nature of the chronic fluid and lipid exudates, the contents of the large cystoid spaces were drained through a retinotomy using a soft-tipped aspiration cannula. Postoperative follow-up after surgical drainage showed immediate resolution of the macular cystoid spaces and gradual resolution of the dense subfoveal exudates over 1 year. Although surgical intervention led to the eventual resolution of the macular edema and exudates, visual recovery was limited by the chronicity of the condition. Conclusions: Surgical drainage of large macular cystoid spaces in Coats disease can be achieved in eyes that are refractory to medical management. Earlier surgical intervention in select cases may allow for visual rehabilitation.