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Background Acute appendicitis is one of the most common causes of acute abdominal pain. Differences in the management of this large group of patients has important consequences for the patients and the healthcare system. Controversies regarding the understanding of the natural course of the disease, the utility of new diagnostic methods, and alternative treatments have lead to large variations in practice patterns between centres. These national guidelines present evidence-based recommendations aiming at a uniform, safe and cost-efficient management of this large group of patients.Method A working group of six experts with broad clinical and research experience was formed. Additional expertise from outside was consulted during the process. A national survey revealed significant variations in the management of patients with suspicion of appendicitis. The evidence provided in published guidelines and reviews were extracted and systematically graded, according to the GRADE methodology. This was supplemented by additional more recent and more directed search of the literature. Patients treated for appendicitis were involved through interviews. The guidelines were reviewed by external experts before the final version was determined.Results The guidelines cover an extensive number of issues: pathology, epidemiology, aetiology, natural history, clinical and laboratory diagnosis, diagnostic scoring systems, diagnostic imaging, treatment, nursing care, follow-up, quality registers and quality indicators, among others. Special considerations related to children and pregnant women are covered.Conclusion These national guidelines present an extensive and thorough review of the current knowledge base related to appendicitis, and provide up-to-date evidence-based recommendations for the management of this large group of patients.