Objective To investigate the relationship between clinical manifestations and pathological changes of primary IgA nephropathy with segmental glomerulosclerosis in children.Methods Clinical data of children diagnosed with primary IgA nephropathy by renal biopsy at the Department of Nephrology, Children's Hospital of Nanjing Medical University from January 1, 2018 to October 1, 2024 were retrospectively analyzed.. Patients were divided into the non-segmental glomerulosclerosis (S₀) and the segmental glomerulosclerosis (S₁) groups according to the Oxford classification of kidney pathology. The clinical and pathological data of the two groups of children were compared. Spearman correlation analysis was used to analyze the correlation between segmental glomerulosclerosis and various indicators； univariate and multivariate binary Logistic regression analyses were performed to identify influencing factors for segmental glomerulosclerosis.Results 145 patients with IgA nephropathy included 43 females (29.66%) and 102 males (70.34%), with a male to female ratio of 2.37 : 1. In the S₁ group, the body mass index (BMI), incidence of hypertension, 24-hour urinary protein excretion (24 h-UP), urinary microalbumin (mAlb), urinary immunoglobulin G (IgG), urinary albumin-creatinine ratio (ACR), and urinary protein-creatinine ratio (UPCR) were significantly higher than those in the S₀ group (all P < 0.05). Pathologically, the Lee's classification grade in the S₁ group was significantly higher than that in the S₀ group (P < 0.05), and the incidences of mesangial cell hyperplasia and crescent formation were also significantly higher in the S₁ group (both P < 0.05). Spearman correlation analysis showed that segmental glomerulosclerosis was positively correlated with BMI (r_s = 0.176, P = 0.035), BP (r_s = 0.225, P = 0.006), 24 h-UP (r_s = 0.165, P = 0.047), mAlb level (r_s = 0.284, P = 0.000), urinary IgG level (r_s = 0.213, P = 0.010), ACR level (r_s = 0.220, P = 0.008) and UPCR level (r_s = 0.194, P = 0.020). Multivariate Logistic regression analysis revealed that hypertension [O^R = 2.227 (95% CI: 1.115, 4.448) ] was an independent risk factor for segmental glomerulosclerosis (P < 0.05).Conclusions Children with segmental glomerulosclerosis are often complicated with higher BMI, blood pressure, and proteinuria levels, as well as more severe pathological damage. Hypertension is an independent risk factor for segmental glomerulosclerosis. Strengthening blood pressure and proteinuria monitoring in children with segmental glomerulosclerosis has clinical significance.