Inflammatory bowel disease (IBD) is characterized by chronic, relapsing inflammation of the gastrointestinal (GI) tract. In recent years, the incidence is increasing in the pediatric population. Insufficient recognition of extraintestinal manifestations (EIMs) of IBD clinically may delay diagnosis. Therefore, improving the recognition of EIMs of IBD has an important significance in its early diagnosis and treatment. The pediatricians should be alerted that renal lesions of EIMs in IBD are mainly immunoglobulin A (IgA) nephropathy and interstitial nephritis.