Results: In our study, there were 3 women and one man. The mean age at diagnosis was 47.5 years old. Two patients presented with painless hematuria as initial symptom, one with flank soreness and the other one was incidentally found by abdominal sonography. The tumors were all located in the right kidney and were measured with mean diameter of 4.8cm. Two of our patients presented with lymph node metastasis at time of diagnosis, and one eventually developed carcinomatosis and multiple lung metastasis. One patient was diagnosed as stage I, two patients with stage III and the other one with stage IV (liver metastasis) disease. Treatment included two laparoscopic radical nephrectomy, one open nephrectomy and one open partial nephrectomy. In three of our patients, one received Interferon-Alfa-2a injection for one year, one received oral sunitinib treatment for one year and one was included in clinical trial with Torisel one month. Three patients showed no evidence of disease recurrence during mean follow-up of 3.3 years. One patient died after 7 months of follow up due to cancer progression. One patient with liver metastasis at diagnosis received radical nephrectomy, liver tumor resection and 3 months sunitinib treatment and remained no evidence of tumor recurrence after three years follow up.