Objective: To retrieve, evaluate and summarize the best evidence for disease management in children with primary nephrotic syndrome. Methods: The relevant literatures on the management of primary nephrotic syndrome in children were systematically searched in comprehensive databases, relevant guidelines websites and professional society websites at home and abroad. The search time was from the establishment of the database to January 2025. Two researchers independently completed the quality evaluation, evidence extraction and integration of the literature. Results: A total of 16 articles were included, including 4 evidence-based guidelines, 5 expert consensuses, 1 systematic review, and 6 clinical decisions. A total of 34 best evidences were summarized and integrated, including management objectives, medication management, urine monitoring, diet management, infection prevention, lifestyle and recurrence assessment. Conclusion: This study summarizes the best evidence of disease management in children with primary nephrotic syndrome, and provides evidence-based basis for clinical medical staff to implement disease management and health education for children. Clinical medical staff should fully combine the clinical situation and the actual situation of the child when selecting evidence, transform it and apply it to clinical practice.