Introduction: Uveal effusion syndrome (UES) is a rare ocular disease causing idiopathic uveal effusion, often with associated ciliochoroidal and retinal detachment. UES diagnosis is challenging because of overlapping features with other ocular inflammatory, neoplastic, iatrogenic, and drug-induced causes of uveal effusion. While several successful surgical treatments have been described, such as fullthickness or partial-thickness sclerectomy, medical therapies may also have a therapeutic role.Objective: To provide an updated review of the published literature on the course of the disease, medical and surgical management strategies, as well as newer treatment modalities.