en Pediatrics Case Report and Review of Literature Case Report and Review of Literature <strong>Background</strong>: Incontinentia pigmenti (IP) is a rare X-linked dominantly inherited genetic skin disorder in which most male infants cannot survive. The clinical manifestation is mainly characterized by a characteristic rash, which may also involve multiple extracutaneous organs.&nbsp;<br> <strong>Case Presentation</strong>: We reported three children with pigmentary incontinence, and a literature review was conducted to elaborate on the clinical manifestations of various systems in patients with IP.<br> <strong>Conclusions</strong>: IP is a disease that involves multiple systems and patients with IP are likely to develop serious ocular and neurologic complications. Once diagnosed, neurologists, ophthalmologists, and dentists must consult and evaluate patients multidisciplinaryly. Early intervention and adherence to lifelong follow-up are needed. Incontinentia pigmenti, Retinopathy, Neonatal, Diagnosis, Screening, Follow-up 359 368 http://jpr.mazums.ac.ir/browse.php?a_code=A-10-1212-2&slc_lang=en&sid=1 Yunfeng Zhang zhangyunf@jlu.edu.cn 100319475328460011937 100319475328460011937 Yes Department of Neonatology, The Second Hospital of Jilin University, Changchun, China. Yuan Wang 465098856@qq.com 100319475328460011938 100319475328460011938 No Department of Neonatology, The Second Hospital of Jilin University, Changchun, China. Jinpu Zhang 853614920@qq.com 100319475328460011939 0009-0004-7142-1141 No Department of Neonatology, The Second Hospital of Jilin University, Changchun, China. Na Song 553835899@qq.com 100319475328460011940 100319475328460011940 No Department of Neonatology, The Second Hospital of Jilin University, Changchun, China.