Bilateral nephroblastoma is a rare malignant tumor which is considered to be a hereditary disease. Specific proportion of bilateral nephroblastomas is 5—10% of all renal malignant tumors in children. From 1964 t 2007, 92 children with bilateral nephroblastomas received treatment in the surgical department of thoracoabdominal oncology of Research Institute of Pediatric Oncology and Hematology of N.N. Blokhin Russian Cancer Research Center. Diagnosis was confirmed by morphologic study in all patients. Our study enrolled 83 children, because in remaining 9 patients early dissemination of disease was revealed and they died from disease progression without being treated. Depending upon strategy and tactic of the treatment all patients were subdivided into 2 groups of historic control: first group included patients treated from 1964 to 1979, and second group—from 1980 to 2007. Clinical manifestations of disease and diagnostic algorithm are analyzed. The issues of surgical tactics, pre and post-operative treatment and radiotherapy are discussed. Overall survival rate of children of 2nd group was 79,2%, which is 3 times higher than outcomes in patients who received treatment from 1964 to 1979. Disease free survival rate in patients with bilateral nephroblastoma of 2nd group was 70,2%.