The frequency of congenitally bicuspid aortic valves in patients having cardiac transplantation (CT) is unknown. We reviewed 243 explanted hearts in patients having CT at Baylor University Medical Center, Dallas from June 1997 through November 2011 to determine the frequency of a bicuspid aortic valve in this population. Of the 243 explanted hearts, 7 (2.9%) were found to have a congenitally bicuspid aortic valve: 3 had severe aortic valve stenosis and before CT had had the aortic valve replaced; the other 4 had normally functioning bicuspid valves and underwent CT for cardiomyopathy (ischemic in 1, idiopathic in 2, and hypertrophic in 1). Review of previously published reports of CT and aortic valve disease disclosed that 4 patients had had aortic valve replacement (AVR) from 2 to 8 years before CT, 3 had AVR or aortic valve repair of the donor heart at the time of CT, and 4 had AVR or transcatheter aortic valve implantation from 1 to 14 years after CT. Some of these aortic valve replacements, before, at the time of, or after CT were in patients with congenitally bicuspid aortic valves. In conclusion, congenitally bicuspid aortic valves were found in 7 of 243 explanted hearts in patients having CT at a single medical center in a 14-year period: 4 had functioned normally and 3 were severely stenotic. Previous reports of patients having AVR or repair before, during, and after CT were reviewed.