Abstract

Purpose: This study investigated the dental disease patterns and healthcare utilization of pediatric patients with rare diseases in Korea using national and clinical data. Materials and Methods: A comprehensive analysis was conducted on substantial data obtained from the Health Insurance Review and Assessment (HIRA) Service and clinical records from Jeonbuk National University Dental Hospital, focusing on patients between the ages of 0 and 19 who had been diagnosed with rare diseases during the period spanning from April 2019 to April 2024. Results: Analysis of HIRA data (387,838 patients) indicated that 61.9% of patients were aged 0–9 years. Disorders of tooth de velopment and eruption accounted for 91.2% of care. The most common diagnoses were dental caries (33.7%) and disorders of tooth development and eruption (25.3%). Total medical expenses and out-of-pocket costs both increased annually. Data from Jeonbuk National University Dental Hospital revealed that patients with congenital malformations and nervous system disorders required frequent long-term management involving hygiene, restorations and extractions. Conclusions: Although the dental disease spectrum mirrors that of the general population, management is com plicated by systemic conditions. Given the heavy reliance on primary care, specialized education for local prac titioners is crucial. In order to improve quality of life, it is essential to establish primary treatments, expand insurance coverage for essential non-covered treatments and create a robust referral system between specialized centers and local clinics.(J Korean Dent Assoc 2026; 64(4): 111-120)