Tehran University of Medical Sciences Frontiers in Dentistry 2676-296X 13 4 2016 12 31 295 301 EN Rezvan Rafatjou Assistant Professor, Department of Pediatric Dentistry, Faculty of Dentistry, Hamadan University of Medical Sciences, Hamadan, Iran Fariborz Vafaee Associate Professor, Dental Research Center, Department of Prosthodontics, Faculty of Dentistry, Hamadan University of Medical Sciences, Hamadan, Iran Hanif Allahbakhshi Assistant Professor, Department of Prosthodontics, Faculty of Dentistry, Kashan University of Medical Sciences, Kashan, Iran Porousha Mahjoub Assistant Professor, Department of Pediatric Dentistry, Faculty of Dentistry, Guilan University of Medical Sciences, Rasht, Iran 2016 06 07 2016 08 27 Incontinentia pigmenti is a rare inherited disorder involving abnormalities of the skin, hair, eyes, musculoskeletal system, central nervous system, and the teeth. Dental abnormalities are the most common manifestations of this disorder. The purpose of this case report was to present the clinical and radiological findings of a seven-year-old girl as well as the results of her five-year follow up. The patient showed faded linear pigmented macular lesions on the trunk and on upper and lower limbs. Dental examination was notable for conical and peg-shaped anterior teeth as well as delayed eruption of primary and permanent teeth. In addition to conservative treatments, prosthetic treatments such as interim removable partial dentures were indicated for the patient. https://fid.tums.ac.ir/index.php/fid/article/view/1295 https://fid.tums.ac.ir/index.php/fid/article/download/1295/904