The aim of this study was to evaluate if health and oral health status of children and adolescents with different stages of CKD are associated with their health-related quality of life (HRQoL), oral health-related quality of life (OHRQoL) and socioeconomic and demographic conditions. One hundred children and adolescents with CKD were age and gender matched to 100 individuals without CKD (mean age ± SD = 13.04 ± 2.57).Oral health was characterised by means of gingival bleeding index (GBI), plaque index (PI), the decayed, missing, and filled teeth(DMFT) index and the developmental enamel defect (DED) index. All children and adolescents answered two Peds QL® instruments (general and oral health scales). Comparing the mean scores of HRQoL and OHRQoL between groups, we observed that CKD group demonstrated worse perceptions when compared to non-CKD group. Multiple linear regression analysis with bootstrap estimation of variance (1000 replications) showed association between dental caries experience (p < 0.001), gingival inflammation (p < 0.001) and diagnosis of CKD (p = 0.027) with the OHRQoL and between physical and the emotional domain of HRQoL, when moderate/severe gingival inflammation and hypoplasia were present. The implementation of public policies that contemplate the early dental preventive intervention in CKD children and adolescents should occur aiming to improve their oral health, once oral manifestations can directly affect the aspects of the HRQoL and OHRQoL of these individuals.