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Background and Objective: The true prevalence of the extraintestinal manifestations (EM) associated with inflammatory bowel disease (IBD) may vary depending on the geographic area, IBD population, location and duration of the disease, medication and diagnostic accuracy. The aim of this study was determine the prevalence of the major EM of IBD and their differences between Crohn's disease (CD) and ulcerative colitis (UC)., Patients and Method: A prospective study with a total of 566 patients (295 CD with median follow up 11.6 years [range: 2-32 years] and 271 UC with median follow up 10.4 years [range: 2-36 years]. Data related to the clinical course, EM and laboratory tests were obtained at diagnosis and during follow-up., Results: EM related with IBD appeared al least once in 46.6% of the patients. Joints manifestations were the most common EM. The EM were equal frequent in UC (51.5%) as in CD (42.2%). Hepatobiliary manifestations (odds ratio [OR] = 1.91; 95% confidence interval [CI] 1.15-3.16; p = 0.007), venous thromboembolism (OR = 4.26; 95% CI, 1.3-15.4; p = 0.006) and arthralgias (OR = 1.59; 95% CI, 1.01-2.5; p = 0.035) were more frequent in UC than CD. Erythema nodosum (OR = 2.35; 95% CI, 1.13-5.0; p = 0.013) and peripheral arthritis (OR = 1.95; 95% CI, 1.02-3.74; p = 0.029) were more frequent in CD. The prevalences of ocular, and the rest of joint manifestations were not different according to UC or CD., Conclusions: Prevalence of EM in Spanish IBD patients is among the highest ever reported. The distribution of the EM observed is different between CD and UC. It is necessary to know to allow to prompt diagnosis and prevent undesirable complications.