Background Initial misdiagnosis of cytomegalovirus retinitis (CMVR) may lead to irreversible loss of vision and systemic deterioration. We retrospectively reported some misdiagnosis related to CMVR. Methods The medical records of 92 consecutive patients diagnosed or misdiagnosed as CMVR were reviewed retrospectively at the ophthalmology department of Beijing youan hospital from July 2017 to October 2019. The primary outcome measure was to evaluate cases with CMVR who were initially misdiagnosed or who were misdiagnosed as CMVR. Results In 8 (8.7%) out of the 92 patients, the initial diagnosis was incorrect. The median age of the eight patients was 37.5 years (range 20-46 yeas). All (7/7, 100%) patients were male. Patients with CMVR were initially misdiagnosed as diabetic retinopathy (1/92,1.1%), branch retinal vein occlusion (1/92,1.1%), ischemic optic neuropathy (1/92,1.1%), Behcet′s disease (1/92,1.1%), iridocyclitis (2/92, 2.3%), and progressive outer retinal necrosis (1/92,1.1%). One patient with binocular renal retinopathy and chronic renal insufficiency was misdiagnosed as CMVR (1/92,1.1%). All patients presented binocular involvement (sixteen eyes), and two patients (four eyes) presented pan-retinal involvement. Fourteen eyes (14/16, 87.5%) had optic disc or macular area involved. One patient is blind, and two patients had a low vision when the diagnosis is finally clear. Five patients had systemic symptoms. Seven patients were finally diagnosed with AIDS showing an extremely low level of CD4 + T lymphocyte: median of 5 cells/ul (range 1-9 cells/ul). Conclusion The misdiagnosis of CMVR can occur in young male patients. The ophthalmologist should pay more attention to CMVR and systemic symptoms insulting to avoid deterioration of vision and delaying in the management of systemic conditions.