This study presents a comprehensive review of the literature regarding the use of urinary neutrophil gelatinase-associated lipocalin (uNGAL) as a diagnostic tool for urinary tract infection (UTI) in children. Meta-analysis was conducted to evaluate the effectiveness of uNGAL in diagnosing UTI and differentiating acute pyelonephritis (APN) from other sites infection in pediatric patients. We searched PubMed, Web of Science, the Cochrane Library and EMBASE for reports published up to January 2023. We only included published literature that addressed the diagnosis of UTI and APN with the use of uNGAL in children aged 0-18 years. Two authors independently reviewed the included studies and extracted the corresponding data according to the inclusion and exclusion criteria. The sensitivity, specificity and area under the curve for each study were pooled by using a bivariate mixed-effects model. A total of 13 studies met the inclusion criteria for this review: 8 reported on uNGAL diagnosis of UTI, 2 on uNGAL diagnosis of APN, and 3 on both UTI and APN. Among all included studies, uNGAL had good sensitivity (0.88, 95% CI 0.79-0.94) and good specificity (0.86, 95% CI 0.78-0.92) for the diagnosis of UTI. The sensitivity and specificity of uNGAL for the diagnosis of APN were 0.79 (95% CI 0.72-0.85) and 0.78 (95% CI 0.50-0.93), respectively. uNGAL has good sensitivity and specificity in the diagnosis of UTI in children and is a promising marker. However, the use of uNGAL still does not provide significant advantages in the diagnosis of APN in children. Consequently, there is a need to optimize and further explore the assay for improved diagnostic accuracy.