A cardiac fibroma was successfully resected from the interventricular septum of a 25-year-old woman. The clinical data were correlated with a review of the data on 144 other patients, thereby providing a clinical profile and management strategy for this type of tumor. Initial manifestations of a fibroma were determined to be congestive heart failure (21%), tachyarrhythmias (13%), and chest pain (3.5%). A majority of patients were asymptomatic (36%) with abnormal physical findings or an abnormal chest roentgenogram. Finding the tumor at autopsy incidentally or on sudden death (23%) indicated the lethal potential. A few (3.5%) of the reports on patients with cardiac fibroma were without clinical data. Noninvasive imaging by echocardiography, computed tomography, and nuclear magnetic resonance improved the diagnosis. Surgical treatment was successful in 53 of the 84 patients for whom it was attempted.