Infections is a common complication of nephrotic syndrome (NS). Our objective was to evaluate the frequency and risk factors for serious bacterial infections (SBI) in febrile children with NS. We reviewed 239 admissions of 107 children with NS who were admitted with fever to a tertiary hospital in Israel, during 1995 to 2016. SBI was diagnosed in 35 admissions (14.6%), most commonly with pneumonia (n = 12), bacteremia/sepsis (n = 8), and urinary tract infection (n = 6). Patients with SBI were more likely to be female (60.0% vs 36.3%, P = .008) and have nephrotic-range proteinuria (71.4% vs 43.6%, P = .010) and edema (62.9% vs 27.0%, P < .001) on admission. No differences were found between the SBI and non-SBI groups in the clinical and histopathological type of NS, immunosuppressive treatment, rate of pneumococcal vaccination, and prophylactic antibiotics. In summary, 1 of 7 children had SBI, most commonly pneumonia, bacteremia/sepsis, and urinary tract infection. Active nephrosis was associated with an increased risk for SBI.