Introduction: Inflammatory bowel disease (IBD) is a disorder of unknown etiology categorized into three groups including Crohn disease (CD), ulcerative colitis [UC], and intermediate colitis (IC). In addition to gastrointestinal (GI) symptoms, childhood IBD frequently present with extra GI manifestations. In present study, we aimed to determine extra GI symptoms in children with IBD in Iran. Methods: Children <18 years old with established IBD diagnosis referred to the Gastroenterology Clinic affiliated with Shiraz University of Medical Sciences during 2007-2017 were included. Results: Eighty-five children were assessed. CD and UC comprised 26 (30.6%) and 47 (55.3%) of the patients. The mean age was 14.09±2.5 years old with 50% of them were boys. The most frequent presenting complaint was rectal bleeding (37.2%). In patients with CD and UC, 30% and 29% of the patients represented at least one extra GI symptom. The most common extra GI manifestations were growth retardation (11.5%) and arthralgia (7.8%) in children with CD and UC respectively. Conclusions: Extra GI symptoms are relatively common in children with IBD. Caution should be taken to avoid confusion with other disorders and to timely manage these manifestations.