Abstract
Collecting duct carcinoma, also known as Bellini duct cancer, is a rare subtype of renal cell carcinoma with a poor prognosis in the metastatic setting. There are limited data to suggest the efficacy of targeted therapy or immune checkpoint inhibitors for collecting duct carcinoma, except for small series and case reports. Herein, we present the case of a patient with collecting duct carcinoma who exhibited a complete response to pembrolizumab and long-term remission approximately 5 years after drug withdrawal.