Purpose. With immunosuppressants being widely used, Pneumocystis jirovecii pneumonia (PCP) has been increasing and could be life-threatening among HIV-negative patients.is study aimed at identifying prognostic factors of PCP in patients with nephrotic syndrome. Methods. We retrospectively investigated patients with nephrotic syndrome who were diagnosed with PCP. e diagnosis of PCP was based on clinical manifestations, radiological findings, and microbiological confirmatory tests. Predictors of outcome were determined with multivariate logistic regression analysis. Results. A total of 57 patients were included in this study. e PCP mortality was 33.3%, which increased to 48.6% if ICU admission was required and to 60% when mechanical ventilation was needed. e T lymphocyte count and CD4/CD8 ratio independently predicted the outcome of PCP, so did the CD4 + T lymphocyte count (OR, 0.981; 95% CI, 0.967-0.996; p � 0.001). e cut-off value of 71 cells/μl for the CD4 + T lymphocyte count was determined to identify patients with poor prognosis. No association was found between PCP mortality and the type of immunosuppressant used. Conclusions. PCP is a fatal complication among nephrotic syndrome patients receiving immunosuppressive therapy. e CD4 + T lymphocyte count is suggested as an independent predictor of prognosis, which can be used clinically to identify patients with high risk of unfavorable outcomes.