Abstract
Leiomyosarcoma arising from renal pelvis is a very rare disease. A patient was admitted to hospital with left renal colic due to nephrolithiasis, with a filling defect in renal pelvis that was considered to be a blood clot. Diagnosis of leiomyosarcoma was made after visual inspection and biopsy of the exophytic lesion. Laparoscopic radical nephrectomy was performed, histological and immunohistochemical investigation confirmed leiomyosarcoma with mixomatoid component. No adjuvant treatment was performed, the patient remains healthy 5 years after surgery without recurrence. Herein we provide literature review, discussion of the diagnosis and treatment scenario of the patient with renal pelvis leiomyosarcoma.