Children with pediatric rheumatic diseases (PRDs) are required to comply with long-term treatment regimens that may not have immediate beneficial effects or may be required primarily to prevent further morbidity. As with other chronic disease regimens, compliance can be a significant problem that interferes with treatment efficacy and adequate clinical outcomes. This paper reviews (1) the prevalence and types of compliance problems in the management of PRDs; (2) patient/family, disease, and regimen factors that may be associated with treatment noncompliance; and (3) strategies for improving compliance with regimens for PRDs.