Iatrogenic Creutzfeldt-Jakob disease (CJD) has never been reported among recipients of dura mater grafts processed in the US. We recently investigated a report of such a case in a 72-year-old man with a typical clinical presentation of CJD. We found no evidence of CJD in either the 34-year-old donor or in other, proximal patients undergoing craniotomies. Although the graft may have caused the illness, sporadic CJD is a more likely explanation, with the graft being coincidental.