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Acute appendicitis is a common occurrence in children. The current trend is to perform appendicectomy once acute appendicitis is diagnosed. The aim of this study was to evaluate the types of pathology seen in appendices removed for acute appendicitis and correlate them with clinical features. The clinical, operative and histological records of 302 children who had appendicectomy at the Jos University Teaching Hospital from 1995-2003 were retrospectively reviewed. There were 123 (40.7%) boys and 179 (59.3%) girls (m: f = 1: 1.5). Their ages ranged from 2-15 years (median: 12 years). The main symptoms and signs at presentation were right lower quadrant abdominal pain, anorexia, nausea, vomiting, right quadrant tenderness, guarding and rigidity. Clinical impressions included simple acute appendicitis in 260 and perforated appendicitis in 42 patients. Of the 302 children who had clinical diagnosis of acute appendicitis, only 144 (47.7%) were confirmed at histology. Lymphoid hyperplasia was the most common pathology seen in the appendix specimens. The clinical impressions of acute appendicitis made by the surgeon agreed with pathologists' report in 63.9% and disagreed in 36.1% of cases. Negative appendicectomy rate was 52.3%. Although appendicectomy is the most commonly performed emergency abdominal surgery in children, the procedure is still associated with a high negative appendicectomy rate. [ABSTRACT FROM AUTHOR]