Akdağ, UğurTozar, Kamile Nur2025-03-092025-03-0920240917-2394https://doi.org/10.1016/j.pdj.2024.11.006https://hdl.handle.net/20.500.12662/4899Congenital pain insensitivity syndrome with anhidrosis (CIPA, MIM 256800) or hereditary sensory and autonomic neuropathy (HSAN) type IV is a rare autosomal recessive disease. CIPA has characteristic symptoms such as failure to respond to painful stimuli, lack of thermal sensitivity, decreased or absent sweating (anhidrosis), varying degrees of reduced intellectual disability, musculoskeletal fractures, and joint deformities. A 4-year-old female patient diagnosed with CIPA syndrome presented to our clinic with the complaint of self-harm with her anterior teeth. In the clinical examination, sores were detected on the child's hands, fingers, mouth, and tongue. The front teeth of the patient, which caused the trauma, were extracted. In the 6-month follow-up, the patient's wounds healed. In this case report, the treatment of a patient with CIPA syndrome who was severely self-harming is described. © 2024 Japanese Society of Pediatric Dentistryeninfo:eu-repo/semantics/closedAccessCongenital insensitivity to pain and anhidrosis (CIPA) syndromeHereditary sensory and autonomic neuropathy (HSAN) type IVLack of pain sensationPediatric patientTooth extractionArticle10.1016/j.pdj.2024.11.0062-s2.0-85212345401Q2