To investigate the retinal vascular abnormalities in both affected and fellow eyes of presumed unilateral Coats disease patients using ultra-widefield fluorescein angiography (UWF-FA) and their association with visual prognosis. A retrospective review of medical records was conducted on 30 patients diagnosed with presumed unilateral Coats disease, who were evaluated with UWF-FA from March 2003 to May 2024 at a tertiary referral hospital. Clinical features and multimodal imaging findings were evaluated, and factors related to final visual outcomes were analyzed. All 30 patients were diagnosed with presumed unilateral Coats disease at presentation, comprising 11 childhood-onset (36.7%) and 19 adult-onset patients (63.3%). Retinal vascular telangiectasia was observed in 51.7% of the fellow eyes. The extent of telangiectasia and exudate in the affected eyes did not significantly correlate with the extent of telangiectasia in the fellow eyes. In the more affected eyes, the childhood-onset group had a significantly greater extent of capillary dropout compared to the adult-onset group (5.0 clock hours vs. 2.8 clock hours, p = 0.023). In the fellow eyes, telangiectasia tended to be more frequent in the childhood-onset group, without statistical significance (63.6% vs. 44.4%, p = 0.160). In the multivariable regression analysis, the final best-corrected visual acuity (BCVA) in the more affected eyes was significantly associated with initial BCVA. The mean extent of telangiectasia in the temporal and nasal quadrants (odds ratio, 12.759; p = 0.043) and the initial BCVA of the more affected eyes (odds ratio, 11.841; p = 0.024) were identified as prognostic factors for final moderate to severe visual loss (Snellen BCVA <20 / 66). About half of the presumed unilateral Coats disease cases exhibited features of the bilateral disease. Visual prognosis is associated with the peripheral retinal telangiectasia in the temporal and nasal quadrants as well as initial BCVA in the affected eyes while it is not associated with retinal vascular abnormalities in the fellow eyes.