Core tip: Cap polyposis is a rare and under-recognized condition with distinct clinical, endoscopic and histopathological features. All children with cap polyposis invariably present with rectal bleeding. Awareness of this diagnosis is important as its clinical and endoscopic features can mimic inflammatory bowel disease resulting in prolonged and inappropriate treatment. This article evaluates the clinicopathological features and treatment outcomes in a series of children with cap polyposis. Complete polypectomy should be performed where possible in combination with medical therapy. Prognosis is good for children with few polyps although recurrence rate is high in those with multiple polyps at diagnosis requiring further surgical intervention.