Introduction Although urothelial carcinoma (UC) generally is non-invasive, contrastingly in 25% of patients UC metastasizes. Isolated CNS metastasis from UC without other distant metastases are considered rare. In this case report we describe a patient with an isolated and solitary cerebellar metastasis from UC. Case presentation A 77-year-old male was diagnosed with a pT2N0M0 high-grade urothelial carcinoma and treated with transurethral resection and chemoradiation therapy. Several months later, the patient presented with neurological symptoms, and radiological imaging revealed a solitary cerebellar mass. A body CT scan showed no other metastasis. After surgical resection, histology confirmed a urothelial origin of the mass, matching his primary UC and the patient received post-operative stereotactic radiotherapy at the surgical site. Conclusions Isolated brain metastases without other distant metastases from UC are rare, so histologic confirmation of the brain metastasis is essential, particularly when the time interval between diagnosis of the UC and brain metastasis increases.