Background Multidrug resistant (MDR) and extensively drug resistant (XDR) Acinetobacter baumannii presents challenges for clinical treatment and causes high mortality in children. We aimed to assess the risk factors for MDR/XDR Acinetobacter baumannii infection and for overall mortality in this patient population. Methods This retrospective study included 102 pediatric patients who developed MDR/XDR Acinetobacter baumannii infection in the pediatric intensive care unit (PICU) of Shanghai Children’s Hospital in China from December 2014 to May 2018. Clinical presentations and outcome of the patients were analyzed. The primary outcome was overall mortality. Secondary outcomes included the 28-day mortality and the length of hospital stay. Results Of the 102 patients (63 males and 39 females; mean age: 51.79 months), the overall mortality rate was 29.41%. 18(17.64%) had bloodstream infections;4(3.92%) for which cerebrospinal fluid (CSF) cultures were positive; 14(13.73%) of them got positive cultures in aseptic fluid; 10 (9.8%) had central catheter-associated bloodstream infections; lower respiratory isolates (56/102) accounted for 54.9% of all patients. Non-survival patients appeared to have a lower NK cell activity 5.87%(2.43%, 8.93%) vs. 8.45%(4.51%, 14.61%), P =0.011), higher CD4 + T cell ratio (35.32% (29%, 47.81%) vs. 31.97% (20.52%, 38.22%), P = 0.045), and higher serum level of interlukin-6 (IL-6, 235.51(0.1, 4172.77) pg/ml vs. 0.1(0.1, 110.92) pg/ml, P = 0.028), interlukin-8 (IL-8, 22.73(3.14, 540.12) vs. 0.1(0.1, 25.85)pg/ml, P = 0.03) , and interlukin-10(10.82(0.1, 83.29)pg/ml vs. 6.05(0.1,21.81)pg/ml) were observed. Multivariate logistic analysis indicated that high serum level of BUN (RR, 1.216, 95%CI, 1.27-2.616; P = 0.001) and high serum level of Cr (RR, 1.823, 95%CI, 0.902-0.980;P=0.004,) were associated with high risk of overall mortality in MDR/XDR Acinetobacter baumannii infected patients. Conclusion MDR/XDR- Acinetobacter baumannii is an important opportunistic pathogen that causes nosocomial infection in PICU with a rather high mortality. The incidence increased in recent years, ineffective management, immune dysfunction, acute kidney injury contributed to the risk of death.