Several recent publications have contributed to our understanding of the processes involved in development of the Mullerian ducts in both sexes and regression of these structures in male embryos. Additionally, new insights in the regulation of the anti-Mullerian hormone ( AMH) signaling pathway, the pathway, which mediates the male specific degeneration of Mullerian ducts, have been gained. It has become clear that the Mullerian duct is formed by invagination of the coelomic epithelium and elongates primarily by proliferation. Later on cells of the coelomic epithelium perform epithelial to mesenchymal transition and move around the epithelium of the Mullerian duct to induce degeneration of this structure in male embryos. Besides AMH and its specific type II receptor AMHR2 two different type I receptors as well as different SMAD family members have been shown to be involved in the AMH signaling cascade. Other factors including WT1, WNT7a, beta-catenin and MMP2 act upstream and downstream of AMH signaling. Here we try to draw an overall picture of Mullerian duct formation and regression by integrating the recent literature in the field. Copyright (C) 2007 S. Karger AG, Basel.