Bladder tumour is rare in children and usually corresponds to embryonic rhabdomyosarcoma. However one of the differential diagnoses is inflammatory pseudotumour of the bladder which is a benign lesion with a good prognosis. The authors report the case of a 3-year-old boy who presented with macroscopic haematuria related to a lesion of the anterior bladder wall. Histological examination of the partial cystectomy specimen demonstrated inflammatory pseudotumour of the bladder that was confirmed by a review examination. Thirteen cases have been reported in the literature over the last ten years and all had a favourable outcome after complete resection of the lesion. Thorough histological examination of the lesion is therefore important to guide treatment.