a report of incontinentia pigmenti in an 11-year-old girl

Introduction: incontinentia pigmenti (ip) is a rarely diagnosed x-linked dominant disease affecting tissues of ectodermal andmesodermal origin such as cutaneous tissues, teeth, eyes, hair, and the central nervous system. dermatologic manifestations areoften the first signs observed in patients diagnosed with ip and are present in nearly all the subjects, but they are less harmful anddo not require treatment. oral manifestations in patients diagnosed with ip might affect both the deciduous and permanent teeth,with tooth shape anomalies and hypodontia, delayed tooth eruption, cleft palate, and high arched palate. these oral abnormalitiesinfluence feeding, quality of life (qol), and self-esteem of the patient but can be successfully corrected by oral rehabilitation.case presentation: here, we report the case of a female patient, aged 11 years, presenting with dental manifestations such as hypodontia,conical teeth, delayed tooth eruption, narrow and atrophic dental ridge, and also some non-dental findings of ip. herdental management included oral hygiene instructions, extraction of all unrestorable primary molars, and composite filling of allprimary canines.conclusions: a removable space maintainer was constructed for the patient, which resulted in favorable esthetic outcomes, properre-establishment of mastication, and improved self-esteem.