The emergence of variant CJD (vCJD) in the last decade has heightened awareness of the need for rigorous infection control precautions in all healthcare environments. It has also raised particular problems in relation to the clinical management of patients who are either suffering from prion diseases or who are perceived as being 'at risk'. From the healthcare perspective CJD is of concern because at present it is an incurable, fatal disease and the causative agent, abnormal prion protein, is resistant to conventional inactivation procedures. Many healthcare workers are concerned about the risks of cross-infection when treating such patients. There are a number of guidelines for the management of CJD patients, though the information they provide sometimes appears contradictory and may require some interpretation in the clinical setting. This paper is based on real-life case scenarios and the advice given to dentists for the management of a group of patients with different manifestations of prion diseases.