Purpose:The objective of this study is to perform a systematic review and provide an update on the diagnosis and management of retinopathy associated with incontinentia pigmenti (IP).Design:This is a systematic review.Methods:A literature search was performed for articles related to IP-associated retinopathy on PubMed, Embase, and ScienceDirect with no date limitations. Articles published on ophthalmology journals were screened and original human studies pertaining to the diagnosis and management of IP retinopathy were included for data analysis. The clinical and imaging findings, treatment, follow-up frequency and duration, and final visual outcomes were recorded.Results:The literature search retrieved 388 articles across the databases. Forty-two full-text peer-reviewed original human studies, containing 100 eyes of 60 unique patients of IP retinopathy, met our inclusion criteria. The majority of patients (68%) presented with IP retinopathy during the first 2 years of life, and 12 eyes of 8 patients who progressed from mild to severe IP retinopathy were all presented before the age of 4 months. Thirty-one (31%) eyes developed retinal detachment, 24 (24%) eyes had a final visual acuity of light perception or no light perception, and 14 (14%) eyes had a visual acuity of 20/50 or better.Conclusion:The results of this systematic review suggest that early detection and early frequent reevaluation of retinopathy in patients with IP in the first 2 years of life are key in obtaining optimal visual outcomes. Therefore, we propose an updated IP retinopathy management algorithm.