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Abnormal Red Reflex: Etiologies in a Pediatric Ophthalmology Population. Children who present with an abnormal red reflex (ARR) are often referred to ophthalmology due to concern for retinoblastoma. However, an ARR can indicate a wide variety of pathologies, all of which have the potential to develop amblyopia and irreversible vision loss. In this retrospective cohort study, we demonstrate that children who presented with an ARR had a mean age of 22.0 ± 32.5 months and were more frequently referred by their pediatricians (74.5%). The majority of these patients (61.8%) had a normal examination on further evaluation, followed by refractive error (20.4%). Amblyopia was diagnosed in 83.9% of patients with refractive error, with a mean age of 50.3 ± 49.2 months. Because many ARR-associated pathologies require time-sensitive treatment to prevent vision loss, proper screening is critical for diagnosis. Pediatricians play a key role in screening, so education on more common ARR pathologies can better facilitate referrals and improve outcomes.