Purpose: The aim of this study was to investigate the clinical features and the frequencies of extraintestinal menifestations (EIMs) in pediatric inflammatory bowel disease (IBD) and to see the related factors with the development of EIMs. Methods: The medical data of the IBD patients were reviewed retrospectively from June 2010 to July 2017. Baseline demographic findings, Paris classifications, Pediatric Crohn’s Disease Activity Index ( PCDAI) and Pediatric Ulcerative Colitis Activity Index ( PUCAI) scores, CRP, ESR, fecal calprotectin, BMR and the specific diagnosis of EIMs were investigated. The secondary extraintestinal complications and the complications of medication were excluded. Results: Total of 172 patients was enrolled, of whom 138 (80.2 %) were Crohn’s disease (CD), 34 (19.8 %) were Ulcerative colitis (UC). The mean age at the diagnosis was 13.65±3.03 years for CD, 12.71±3.11 years for UC, and the mean follow-up duration was 4.05±2.88 years for CD, 3.27±2.28 years for UC. The EIMs occurred in 40 patients (22.1%), which were consisted of 34 (85%) in CD and 6 (15.0%) in UC, respectively. EIMs were arthritis or arthralgia (n=15, 37.5%), Stomatitis and/or oral ulcer (n=10, 25%), hepatitis (n=5, 12.5%), pancreatitis (n=2, 5%), nephritis (n=4, 10%), erythema nodosum (n=2, 5%), ankylosing spondylitis (n=1, 2.5%), and pyoderma gangrenosum (n=1, 2.5%). The Paris classification for CD did not show any significant difference between EIM and non-EIM group, but the one for UC showed a significant difference in severity (p=0.032). The age at the initial diagnosis of each group showed a significant difference in CD (p=0.003), not in UC, between the EIM and non-EIM groups. The PCDAI and PUCAI scores were not significantly different between the EIM and non-EIM groups, in CD and UC, respectively. Conclusion: The EIMs were not significantly related with the disease location or extent in the pediatric IBD patients. The initial diagnosis age in CD and the disease severity of UC were significantly related with the occurrence of EIMs.