Objective To investigate the clinical features and prognostic risk factors of pneumocystis pneumonia (PCP) in patients with glomerular disease. Methods The medical charts of all patients with confirmed PCP, diagnosed in Peking University First Hospital from August 2006 to February 2018 were retrospectively reviewed, and 36 cases with glomerular disease were enrolled. Clinical and imaging data were collected and analyzed. Thirty-six patients were divided into survival group and death group. The clinical data, baseline estimated glomerular filtration rate (eGFR), mechanical ventilation and APACHE II score were compared. Results A total of 27 males and 9 females were included, with age of (49.6 ± 17.5) years. All patients were receiving immunosuppressive therapy at the PCP onset, with a median duration of 2.5 months, and none of them was receiving PCP prophylaxis. The main clinical manifestations included fever (100.0% ), dyspnea (75.0% ) and dry cough (61.1% ). Hypoxemia occurred in 97.2% of patients and 17 cases presented as type 1 respiratory failure. Fifteen out of 30(50.0%) patients had CD4+ T cell counts below 200 cells/mm3. Ground glass opacity was the most common finding in CT imaging of 28 patients, followed with grid shadows, consolidation and nodules. Thirty-five patients received trimethoprim-sulfamethoxazole (TMP-SMX) as initial therapy, and 17.1% (6/35) of them developed acute kidney injury due to sulfonamide use. Ten patients died during hospitalization, with respiratory failure as the only direct cause of death. Elder age, delayed diagnosis of PCP, mechanical ventilation and high APACHEⅡscores were associated with poor survival. Conclusions PCP is a severe complication of immunosuppressive therapy in patients with glomerular disease. Early diagnosis and prompt treatment are critical to improve prognosis. Hydration prior to sulfonamide treatment and alkalization of urine are necessary to reduce the incidence of acute kidney injury.