The Wilms' tumor 1 (wt1) gene is one of at least three genes that are involved in the development of Wilms' tumor, a pediatric kidney cancer. The expression pattern of the gene indicates that wt1 not only plays a role during kidney development but is also involved in the development and homeostasis of several other tissues. The physiological function of the gene, however, remains to be elucidated. The gene products have been implicated in many processes like proliferation, differentiation, and programmed cell death (apoptosis). The WT1 proteins function as transcription factors but may additionally be involved in splicing. Disruption of these activities may lead to aberrant development. In this paper we will discuss the role of the wt1 gene during normal development and homeostasis of several tissues. In addition, we will address the involvement of the gene products in processes like apoptosis and tumorigenesis.