Angiosarcoma is a very rare tumor. The malignancy is high grade and the prognosis is extremely poor. A 51-year-old man was admitted to our hospital with the main complaint of asymptomatic macroscopic hematuria. Since right ureteral cancer was suspected by the imaging examination, laparoscopic right total nephroureterectomy was planned. However, strong adhesion was found between the tumor and the surrounding tissue, and the tumor could not be completely resected from the distal ureter. Pathological diagnosis was primary ureteral angiosarcoma, and staging was right middle ureteral angiosarcoma T3N0M0. However, since surgical findings strongly suspected that the peeled surface was positive, adjuvant radiation therapy was added. He is alive without disease recurrence at one year and eight months after surgery.