The giant kidney worm, Dioctophyma renale infection is a common, widely distributed parasite of mink and some other carnivorous animal but very rarely reported in human. A 57 years old man, visited our department because of left lower quadrant pain and hematuria, foreign body like earthworm. The foreign body is identified as Dioctophyma renale. Computed tomography showed the irregular shape mass in the renal pelvis and upper ureter. We performed radical nephroureterectomy with bladder cuff excision and the mass was pathologically confirmed as the malignant fibrous histiocytoma and not contained any egg or parasite.