An atypical presentation of Coats' disease is reported with a prominent subfoveal nodule with peripheral retinal exudates. A 6-year old boy presented with 6/120 vision in the left eye associated with an elevated 1 mm subfoveal, circular lesion with peripheral exudates. The fluorescein angiogram showed peripheral retinal telangiectasias with leakage consistent with Coats' disease. The prominent subfoveal nodule is an uncommon initial presentation of Coats' disease and physicians should be aware of this atypical finding.