Background: Neonatal appendicitis (NA) is rarely diagnosed due to lack of specific symptoms and signs. The aim of this study is to share our experience with NA.Methods: We performed a retrospective data review from electronic medical records of patients admitted at Children’s Hospital of Chongqing Medical University (CHCMU) with the clinical diagnosis of NA from October 2001 to July 2018.Results: Of 31 neonates, male neonates (n=20) accounted for 64.5%. Abdominal distension and anorexia were the most common clinical features (61.3%, n=19). Neonates with elevated C- reactive protein (CRP) accounted for 92.6% (n=25) where as 51.6% (n=16) had normal white blood cell count (WBC). Of 29 neonates who underwent surgery, 69% (n=20) had perforated appendicitis.Conclusion: Neonatal appendicitis lacks specific clinical features, and early diagnosis is difficult. Clinicians need to have high index of suspicion when attending neonates with features of abdominal sepsis.