Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 51 11 2013 11 15 805 810 EN Maryam Azizzadeh Department of Dermatology, Semnan University of Medical Sciences, Semnan, Iran. Morteza Rezaei Department of Pediatrics, Semnan University of Medical Sciences, Semnan, Iran. Nargess Hashemi Department of Pediatrics, Semnan University of Medical Sciences, Semnan, Iran. 2015 10 11 Incontinentia Pigmenti (IP) is a rare X-linked dominant disorder with skin, eye, central nervous system (CNS) and tooth abnormalities. According to the reported cases, it is estimated that there have been nearly 900-1200 affected individuals. In this article, the literature is reviewed and a case of IP with characteristic skin lesions and optic atrophy is presented. https://acta.tums.ac.ir/index.php/acta/article/view/4344 https://acta.tums.ac.ir/index.php/acta/article/download/4344/4492