Purpose: This work describes a unique clinical feature in Coats disease. Methods: A retrospective series of 2 cases is reported. Results: Two pediatric patients receiving treatment for Coats disease were included. In both cases, vision worsened secondary to paradoxically increased exudation and macular star formation following standard treatment with intravitreal bevacizumab, sub-Tenon triamcinolone acetonide, and laser photocoagulation. After serial treatments under general anesthesia, the exudates in both cases consolidated. Conclusions: A paradoxical exudative retinopathy can occur in some patients when initiating standard treatment of Coats disease. Longitudinal follow-up with continued intravitreal antivascular endothelial growth factor agents, laser photocoagulation, and corticosteroids might help control persistent exudation in these cases.