Background: The relationship between hematuria, a typical presentation of immunoglobulin A nephropathy (IgAN), and long-term adverse prognosis of these patients is still controversial. This meta-analysis aims to clarify the effect of hematuria on renal outcomes in IgAN. Methods: Observational cohort studies reporting associations between various forms of hematuria and renal outcomes among IgAN patients were identified from the PubMed and Embase databases. The pooled adjusted risk ratios (RRs) were computed with random effects models. Results: Thirteen studies encompassing 5660 patients with IgAN were included. Patients with initial hematuria did not have a significantly increased risk of developing end-stage renal disease (ESRD) compared with those without hematuria (RR, 1.32; 95% CI, 0.87-2.00; p ¼ .19). However, initial microscopic hematuria was associated with an 87% increase in the risk of ESRD (RR, 1.87; 95% CI, 1.40-2.50; p < .001), while macroscopic hematuria was associated with a 32% decrease in the risk of ESRD (RR, 0.68; 95% CI, 0.58-0.79; p < .001). Additionally, persistent hematuria might be an independent risk factor for ESRD or a 50% decline in eGFR. Conclusions: Among IgAN patients, hematuria, including initial microscopic hematuria and even persistent hematuria, was possibly associated with renal progression and ESRD. However, independent of other classical predictors, initial macroscopic hematuria might be a protective factor for IgAN.