Wilms’ tumor in adults is rare and preoperative diagnosis may be difficult. Tumor stage and histology are the main determinants of outcome. Adult patients usually present with more advanced disease and the prognosis is poorer than for children. Data concerning therapy are conflicting. A multimodal procedure regardless of the stage with radical nephrectomy, chemotheray with actinomycin D, vincristine and adriamycin, and radiotherapy to the renal bed has often been recommended. However, encouraging treatment results could also be achieved by different and less aggressive treatments depending on tumor stage. Recurrencies are common in advanced disease and usually affect the lungs. Salvage chemotherapy (cisplatin/etoposide) and pulmonary radiation has been effective in some cases. Experimental approaches such as allogenic bone marrow transplantation deserve further consideration.