Purpose: The purpose of this study was to report a case of cytomegalovirus retinitis in the setting of dyskeratosis congenita. Methods: Case report and literature review. Results: A 23-year-old woman who had peripheral occlusive retinal vasculopathy and neovascularization as a result of pancytopenia caused by bone marrow failure also developed cytomegalovirus retinitis. She had been diagnosed with dyskeratosis congenita, which had caused the bone marrow failure and pancytopenia. Conclusion: Dyskeratosis congenita is a multisystem disorder that causes bone marrow failure. It can have retinal manifestations in the form of peripheral proliferative retinopathy and retinitis.