Coats-like retinitis pigmentosa is a rare disease demonstrating both features of Coats disease and retinitis pigmentosa. We are reporting a case of a 15-year-old female with no known medical illness who presented with a one-year history of nyctalopia and bilateral painless blurred vision. Fundus examination revealed bilateral optic disc swelling, telangiectasia, and bony spicules. Optical coherence tomography and fundus fluorescein angiography showed cystoid macular edema (CMO). The diagnosis of Coats-like retinitis pigmentosa with CMO was made. She received laser indirect ophthalmoscope photocoagulation and intravitreal anti-vascular endothelial growth factor therapy, but her vision remained refractory. Her diagnosis was subsequently revised to bilateral intermediate uveitis causing CMO and her vision improved to periocular steroid injections. This case emphasizes how critical it is to identify symptoms and diagnose the illness as soon as possible because treating related complications on time can save a patient's sight and provide long-term benefits.
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