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Abstract: Primary nephrotic syndrome is the most common glomerular disease in childhood.The 2025 KDIGO Guidelines integrate the latest evidence to update the diagnosis and treatment strategies for childhood steroid-sensitive nephrotic syndrome（SSNS）and steroid-resistant nephrotic syndrome（SRNS）.In diagnosis，some definitions are revised，and a“confirmation period”is proposed to clarify the diagnosis of SRNS；prognosis is related to the response to glucocorticoids and the frequency of relapses. In treatment，an 8-12-week regimen of initial glucocorticoids is recommended，with individualized use of glucocorticoids during infections. For frequently relapsing/steroid-dependent nephrotic syndrome（FR/SDNS），combination therapy with steroid-sparing agents［such as calcineurin inhibitors （CNIs），rituximab，etc.］is suggested. For SRNS，genetic testing should be performed as soon as possible，with CNIs as the initial second-line treatment. The outlook indicates that future research should focus on the exploration of shortening the initial course of glucocorticoids，optimizing the selection and dosage of steroid-sparing agents，achieving stratified diagnosis and treatment by integrating genetics and biomarkers，while paying attention to the safety of B-cell depletion therapies and quality-of-life endpoints to promote precision medicine practice.

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