Purpose: To describe the long-term follow-up of an immunocompetent patient with cytomegalovirus (CMV) retinitis. Methods: A single case was analyzed and a literature review performed. Results: A 47-year-old woman presented with a paracentral scotoma in the left eye. Examination showed parafoveal retinitis. An aqueous fluid tap was positive for CMV on polymerase chain reaction; however, there was no recent surgery or local or systemic immunosuppression. A thorough workup, including pan imaging, was unremarkable. The retinitis resolved with oral valganciclovir and adjuvant intravitreal ganciclovir and foscarnet injections. At the 3-year follow-up, the patient’s disease was inactive without development of systemic illness. Conclusions: In patients presenting with retinitis, the diagnostic use of aqueous polymerase chain reaction is critical, especially in atypical cases in which the retinitis might be associated with CMV.