Objective: To investigate the risk factors of IgA nephropathy (IgAN) patients with nephrotic syndrome (NS). Methods: A total of 214 patients of IgAN diagnosed by renal biopsy from January 2012 to December 2017 were retrospectively analyzed. According to whether manifesting as NS in clinic, the patients were divided into NS group and non-NS group. The clinical and pathological characteristics in the two groups were analyzed. Then the clinical, pathological features and outcomes of IgAN with NS as the clinical manifestations were further analyzed. Linear correlation analysis was used to analyze the risk factors related to its prognosis. Results: There were 214 IgAN patients in our study, including 84 patients with NS and 130 patients without NS. There were statistical differences in mean arterial pressure (MAP), hematuria, serum creatinine (SCr), blood urea nitrogen (BUN), blood uric acid (UA), cystatin C (CysC), cholesterol (CHOL), triglyceride (TG) and estimated glomerular filtration rate (eGFR) between the two groups (all P<0.05). The IgAN patients with NS had higher proportion of endocapillary hypercellularity (E1) and cellular or fibrocellular crescents (C1–2) in renal pathology. Furthermore, the proportion of the MESTC scores ≥3, eGFR <60 mL/min at the time of renal biopsy was significantly higher (all P<0.05). In addition, the proteinuria was significantly higher and eGFR was significantly lower at the end of follow-up in the NS group than in the non-NS group (all P<0.05), and the eGFR at the end of follow-up had a significant correlation to the age, MAP, BUN, SCr, CysC, UA, Oxford Classification S and T at the time of renal biopsy in NS group (all P<0.05). Conclusion: The eGFR at the end of follow-up had a significant correlation to the age, MAP, BUN, SCr, UA, S1 and T1–2 at the time of renal biopsy in NS patient.