We have treated surgically 5 patients with renal cell carcinoma in the solitary kidney. The cause of renal absence was nephrectomy for renal stones, in 2 patients and renal tuberculosis, renal cyst and renal hypoplasia in 1 patient each. Four of the 5 patients died. One of the 4 patients died 5 days after surgery due to gastrointestinal bleeding, 1 due to metastasis, 1 due to gastric cancer and one due to hemodialysis complications. Surgical management of renal cell carcinoma of solitary kidney is discussed.