Introduction: Kohlschutter-Tonz syndrome (KTS) is a rare, genetic condition, which typically manifests as a triad of symptoms: 1) amelogenesis imperfecta, 2) infantile onset epilepsy, and 3) intellectual disability. The condition poses dental treatment challenges given the manifestation of amelogenesis imperfecta. Additional considerations are needed to medically manage these patients who present with epilepsy and intellectual disability.
Case Report: Our patient presented with multiple restorative needs, was treated under general anesthesia, and maintained good oral outcomes with close follow-up.
Discussion: To the best of our knowledge, this is the first case report which documents comprehensive dental management of a pediatric patient with KTS.
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