Inflammatory bowel disease (IBD) is a chronic relapsing-remitting inflammation of the bowel, variably associated with extraintestinal menifestations. The etio-pathogenesis of IBD is multifactorial, which includes genetic predisposition, dysregulated immune responses and environmental factors. IBD consists of two main entities i.e. ulcerative colitis (UC) and Crohn’s disease (CD). A temporary diagnosis of IBD unspecified (IBDU) is used when differentiation between UC and CD is not possible. Pediatric IBD (i.e. presenting before the age of 18 years) comprises of up to 25% of IBD patients. CD is twice as common as UC in pediatric IBD. Compared to adults, pediatric IBD may differ phenotypically, in the form of clinical presentation (e.g. growth failure and delayed puberty, which may precede bowel symptoms), as well as in endoscopic and histologic appearances. Variable degrees of chronicity changes and disease activity may be appreciated on histology as a result of chronic inflammation and injury. Assessment for IBD diagnosis on mucosal biopsy, including differentiating between UC and CD (both in pediatric and adult population) requires assessment of multiple histologic components, namely: mucosal architectural distortion, alteration in lamina propria lymphoplasmacytic cell gradient, epithelial abnormalities (e.g. surface erosion, ulceration and metaplasia), neutrophil granulocyte infiltration, distribution pattern of histologic changes, as well as presence/absence of epithelioid granulomas. Making an accurate histologic diagnosis also requires one to be familiar with normal gastrointestinal histology to avoid over/under- or misinterpretation of both abnormal and normal findings. The histology in pediatric IBD may differ from that of adult’s, especially in younger patients (less than 10 years of age). In UC, patients may present with extensive colitis with less severe and less diffuse architectural distortion. There may be patchy inflammation and mucosa may even appear normal or just mildly inflamed at onset. Rectal sparing is seen in about 30% of cases. Some of these features may cause histologic confusion with CD. In Crohn’s disease, there tends to be more colitis (including rectal involvement) than ileitis in younger children. Granulomas and upper GI involvement are also more common as compared to adults. Correct IBD diagnosis, as well as differentiation between CD and UC is pertinent for optimal treatment strategy, and in many instances, requires close correlation of clinical, endoscopic, histologic, and radiologic findings.