Abstract: Objective: To summarize the clinical and imaging characteristics of children with pneumocystis jirovecii pneumonia (PJP) infected by non-human immunodeficiency virus (HIV). Methods: The consecutive cases of PJP with HIV negative confirmed and treated in PICU of Anhui Children's Hospital between August 1st, 2014 and July 31st, 2019 were included. Clinical data of survival and died children after 90 days of admission to PICU were compared. Results: Thirteen cases were analyzed in this study, including 7 males and 6 females， with the onset age of (80.8±23.8) months. Twelve cases had underlying diseases, most of which were primary nephrotic syndrome, congenital immunodeficiency disease and hematologic tumor. Ten cases were treated with immunosuppressive agents before the onset of the disease, and all of 13 cases failed to respond to antibiotics. Clinical symptoms included fever, shortness of breath, cough (mostly dry cough), anorexia /weight loss, and progressive dyspnea. Moans were common in small infants, and moist rales could be heard and fixed in 2 cases. LDH was increased in 13 cases ［(646±122) U·L-1］, and BDG was increased in 8 cases ［(184±119) pg·mL-1］. Chest imaging examination showed that 13 cases had ground-glass opacity in both lungs, and 3 cases had pneumothorax, subcutaneous emphysema and mediastinal emphysema.Compared with the survival group, the lymphocyte count (P=0.012) and oxygenation index (PaO2/FIO2) were lower in the death group (P=0.027), and the time from onset to SMZ treatment was longer(P<0.038). Conclusion: When immunocompromised children have symptoms such as fever, shortness of breath, dry cough, progressive dyspnea, and ground-glass opacity in both lungs by CT, PJP should be considered.