Neurofibromatosis in infants is uncommon and involvement of the bladder is rare. The reported bladder lesions are rare, and in such patients include neurofibromas, neurofibrosarcomas and rhabdomyosarcomas. Neurofibromas can be of different histologic types. The histologic type of the about 20 reported bladder neurofibromas in children is not clarified or is stated to be of plexiform type. We describe herein an unusual case of a neurofibroma of bladder in a child with von Recklinghausen disease. The therapeutic management and the possible prognostic implication of the type of the bladder neurofibroma and bladder lesions other than neurofibroma are discussed.