PURPOSE: We present the first reported case of a condition similar to retinopathy of prematurity in a full-term patient with hypoplastic left heart syndrome. DESIGN: Interventional case report. METHODS: We describe the clinical presentation and surgical treatment of a male baby with severe retinopathy and retinal detachments. RESULTS: The clinical course of this baby was similar to retinopathy of prematurity. The patient had areas of avascular retina, significant retinal neovascularization, fibrous proliferation, and tractional detachment. One eye had a totally detached retina; the other had functional vision after the retina was reattached by several vitreoretinal surgeries, including the use of intraocular silicone oil. CONCLUSIONS: This cyanotic condition can cause a retinopathy with chronic retinal neovascularization and retinal detachments. Permanent surgical retinal reattachment is difficult in the presence of neovascular activity that leads to intravitreous cicatrization.