American Indian/Alaska Native (AI/AN) children experience high rates of dental decay, yet their pediatric oral health-related quality of life (POQL) has not been described. We measured POQL in AI children and compared it in children with reported excellent/very good/good versus fair/poor oral health status (OHS) and assessed association of OHS, child's age, dental service utilization, and dental insurance on POQL scores. Caregivers of 143 AI (100 %), young (mean age 25.1 months) children reported their POQL score as 4.2 (scale 0-100, lower score indicates better POQL); OHS as excellent (35 %), very good (27 %), good (21 %), fair (14 %), and poor (3 %); and utilization of urgent dental services (12 %). Worse POQL was associated with worse OHS (p = 0.01). After adjustment, worse POQL was associated with increased reported use of urgent dental services (p = 0.004). POQL of young AI children was generally favorable but worsened with increased utilization of urgent dental services.