Tehran University of Medical Sciences Frontiers in Dentistry 2676-296X 9 3 2012 09 15 267 70 H Afshar Associate Professor, Dental Research Center and Department of Pediatric Dentistry, Tehran University of Medical Sciences, Tehran, Iran. M Daneshpazhooh A Kiani P Aref Z Baniameri 2015 10 16 Incontinentia pigmenti (IP) is an X-linked dominant genodermatosis characterized by typical skin lesions along Blaschko's lines and associated with ocular, dental, nails, hair, skeletal, central nervous system and cardiovascular anomalies. We report a 5-year-old boy with cutaneous hyperpigmentation along Blaschko's lines, atrophic streaks, strabismus and mental retardation. He showed the characteristic abnormal dentition seen in IP as partial hypodontia, peg-shaped anterior teeth and un-erupted teeth. The expression of IP in boys is exceptional as the disease is lethal in males. https://fid.tums.ac.ir/index.php/fid/article/view/743 https://fid.tums.ac.ir/index.php/fid/article/download/743/718