The brain is a rare site for metastasis in most extracranial pediatric solid tumors, including Wilm’s tumor. Outcome for these patients are generally dismal. Very few cases have been reported to have good survival even after therapy. This paper reports a case of stage IV Wilm’s tumor with lung metastases in a patient who developed a solitary brain metastasis five months after completion of chemotherapy. She underwent resection of the brain tumor followed by chemotherapy and radiotherapy. The patient was alive at the 20-month follow up after diagnosis of brain metastasis and was considered to have radiologically stable disease.