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Incontinentia pigmenti (IP) is a rare genetic condition which is X-linked and predominantly affects the structures arising from neuroectodermal origin such as the skin, teeth, eyes, hair, nails and central nervous system. The dermatological manifestations are seen as the first developing signs. The dental manifestations are also commonly seen. Here we discuss a case of a girl in her early adolescence who was diagnosed with a case of IP. The dental features present in this case were typical, including missing permanent teeth, retained deciduous teeth, microdontia and delayed eruption.