Coats disease in a 3-week-old boy.

Coats disease is a rare, idiopathic retinal vasculopathy that predominantly affects males in the first decade of life. We report the case of a 3-week-old boy who presented with atypical rapidly progressing disease suggestive of Coats exudative vasculopathy. The eye developed retinal fibrosis and phthisis bulbi within 4 weeks. Retinoblastoma could not be ruled out, although histopathology after enucleation revealed no retinoblastoma. To our knowledge, this is the youngest case of Coats disease to be reported in the literature. This case highlights the wide variation in the clinical presentation of Coats disease and the difficulty in differentiating it from diffuse infiltrative retinoblastoma.