Twenty-four patients with mitral valve prolapse underwent cardiac catheterization, exercise testing, and exercise 201thallium scintigraphy. Of 10 patients with coronary artery disease, six had abnormal scintigrams. Two of these six had exercise-induced reversible defects, two had defects that persisted during redistribution, and two had both reversible and persistent defects. Of 14 patients with normal coronary arteries, five had negative scintigrams. Of the remaining nine patients, two had exercise-induced defects, and seven (50%) had defects involving the inferior or posterior wall that persisted during redistribution. Possible mechanisms for this latter finding are discussed. In contrast to previous reports, exercise 201thallium scintigraphy was not entirely successful in identifying patients with coronary artery disease in our patients with mitral valve prolapse.