Background: Using the genetic profile of a tumor, clinicians may be able to give insight in to the aggressiveness or indolence of a patient’s disease. The choice to initiate or hold systemic therapy for those with metastatic disease may be aided by knowing which mutations put a patient at an accelerated risk of death from their disease. Identifying genomic markers in clear cell renal cell carcinoma (ccRCC) for patients with metastatic disease at time of nephrectomy may assist in the decision to initiate early systemic therapy even in those with stable clinical disease. Methods: Using available data from The Cancer Genome Atlas (TCGA), The International Cancer Genome Consortium (ICGC), The Cancer Genomics Project Tokyo, Japan and our intuitional database we identified 157 patients who had metastatic disease at time of nephrectomy. We performed statistical analysis to compare the presence of mutations in five recurrently mutated genes (VHL, PBRM1, SETD2, BAP1 and KDM5C) in ccRCC and overall survival. For each gene, Kaplan-Meier estimates for patients with and without mutations were generated and compared using the Log-rank test and Cox proportional hazards regression. Results: Out of the five recurrently mutated genes analyzed, we found that only mutations in BAP1 showed a statistically significant decrease in overall survival for patients who had metastatic disease at time of nephrectomy. Patients without a mutation in BAP1 had an estimated median overall survival of 32.2 months, whereas patients with a mutation in BAP1 had an estimated median overall survival of 15.4 months, HR 1.717 (CI 1.038 –2.839), p-value=0.0326. Conclusions: Patients with mutations in BAP1 show a statistically significant decrease in overall survival compared to those without mutations in these patients with terminal disease. Given these findings early initiation of systemic therapy may be considered even in those with small or stable residual disease.