Collecting duct carcinoma (Bellini duct carcinoma) is a very rare disease with poor prognosis. Primary tumor surgery is not advisable, even though it is the therapy of choice for suspected malignant space-occupying renal masses. Chemotherapy seems to be inferior to new therapeutic concepts based on single case reports. Immunotherapy with nivolumab and axitinib and additive radiotherapy significantly prolongs survival. At the same time, targeted therapy with cabozantinib seems promising. However, due to the short median survival and the side effects of these therapies, a purely supportive approach should be discussed. It should be decided with the patient whether a systemic therapy should be started or whether a palliative, supportive therapy concept offers more quality of life.