Aortic stenosis (AS) is the commonest native valve lesion, affecting 43% of all patients with valvular heart disease. The optimal treatment of severe AS in cancer patients is unknown. The purpose of this study was to assess the impact of aortic valve replacement (AVR) on survival of cancer patients with severe AS. Cancer patients with severe AS seen at our center between January 2001 and April 2007 were identified. Baseline demographics, symptoms, cancer diagnosis, laboratory data, treatment, and outcome were collected. Patient who had AVR were matched with controls who did not have AS. Out of 39,071 echocardiograms performed over the study period, 1,299 had AS (3.3%), of which 50 patients (0.13%) were identified as having severe AS. Thirteen patients (27%) underwent AVR, and 35 were managed medically. Two patients underwent valvuloplasty and were excluded. Survival was significantly longer in patients with severe AS who underwent AVR and was independent of cancer status or presence of metastases. No difference in survival was found between patients who underwent AVR and matched cancer controls. In a multivariable Cox proportional hazard regression analysis, AVR was the only significant predictor of longer survival (adjusted hazard ratio = 0.22, P = .028). Cancer patients with severe AS who underwent AVR had an improved survival, regardless of cancer status. Copyright © 2011 Mosby, Inc. All rights reserved.