Objectives. This study aims to show the clinical value of ischemia-modified albumin (IMA) and Signal Peptide, Complementary C1r/C1s, Uegf and Bmp1–Epidermal Growth Factor Like Domain Containing 1 (SCUBE1) protein in pediatric appendicitis. Methods. Eighty-one pediatric patients hospitalized in the pediatric surgery ward with acute appendicitis and a control group of 62 pediatric patients with unspecific abdominal pain were included in this prospective case-control study. Thirty-nine patients whose pathology specimens confirmed acute appendicitis made up the final appendicitis group. Results. Patients with appendicitis had higher IMA (p<0.001) and SCUBE1 (p<0.001) levels than the control group. In receiver operating characteristic (ROC) analysis, areas under the curve (AUC) were 0.991 (sensitivity=97.4%, specifity=100%, positive likelihood ratio (+LR) infinity, negative likelihood ratio (-LR)=0.03, positive predictive values (+PV)=100%, negative predictive value (-PV)=98.3% for IMA and 0.803 (sensitivity = 89.7%, specificity =64.5%, +LR=2.53, -LR=0.16, +PV=61.4%, -PV=90.9%) for SCUBE1. Conclusions. The present study shows that IMA may be a reliable marker for a more accurate diagnosis of appendicitis. SCUBE1 can be used to exclude diagnosis if used in combination with other laboratory and clinical data.