Purpose: To highlight the possibility of intraocular calcification in Coats disease and evaluate the ultrasound and computed tomographic findings. Method: A 7.5-year-old boy had a 2-week history of unilateral-glaucoma and leukocoria with retinal detachment, suggestive of Coats disease. The possibility of retino-blastoma, however, could not be excluded by ultrasound or computed tomographic examination, which revealed a retinal detachment overlying a subretinal mass with calcification. The blind, painful eye was subsequently enucleated. Results: Histopathologically, there were telangiectatic retinal vessels in a fold of the detached retina peripherally and proteinaceous exudate in the subretinal space containing cholesterol clefts and foamy histiocytes, characteristic of Coats disease. There was also a fibroosseous nodule in the macular area that correlated with intraocular calcification clinically. Conclusion: This case provides the first documentation of ancillary corroboration of intraocular bone formation in Coats disease, which, although rare, is an important consideration in the differential diagnosis of retinoblastoma.