Background: The aim of this study was to analysis the clinical features, risk factors and outcomes of patients with primary nephrotic syndrome (PNS) who developed pneumocystis pneumonia (PCP).Methods: We systematically reviewed medical records from 18 PNS patients with PCP admitted to our hospital from April 2007 to April 2019. A total of 180 cases were randomly selected as controls from PNS inpatients without infection.Results: In PCP patients, the mean age at presentation was 48.5 years, mean duration of prednisone treatment was 3.7 months and mean prednisone dose on admission was 31.3 mg/d, the most common clinical manifestation was fever (100%) and average PaO2 on admission was 59.5 mmHg. Eight patients (44.4%) had coexisting infections, most often was cytomegalovirus (4 patients), 11 patients (61.1%) had ICU admission and 9 patients (50%) had mechanical ventilation. PCP patients had more prednisone, more immunosuppressive therapy, lower CD4+ cell counts and hemoglobin, and higher serum creatinine than those without infections (p<0.05). Logistic regression analysis showed that patients with prednisone usage and lower CD4+ cell counts were more likely to have PCP compared to controls (p<0.05). All patients survived after treatment.Conclusion: PCP was not unusual in PNS patients, and the most important risk factor was a lower CD4+ cell count, but however, these patients had a good outcome after enough treatments.