This book provides clinicians with clear guidance on treatment decision-making in patients with rare kidney cancers. After a brief review on epidemiology, pathology, and biology, each chapter focuses on the multidisciplinary management of a particular tumor subtype using the full range of available cancer therapy modalities, including surgery, radiotherapy, chemotherapy, targeted therapies, and immunotherapy. Emerging therapies and future directions in the management of each cancer subtype are also discussed. The chapters are all written by multidisciplinary teams of international experts comprising at least a urologist, a medical oncologist, and a pathologist. Rare kidney cancers represent 15% of renal cell carcinomas. In comparison with clear cell renal cell carcinomas, little is known about the biology of such cancers, and few trials have reported on the efficacy of targeted therapies in the metastatic setting. Optimal management thus poses significant challenges and often requires a multidisciplinary team. This book will be an ideal reference guide for all clinicians involved in the care of patients with these orphan tumors.