The Hedgehog (Hh) signalling pathway is a highly conserved developmental pathway, which plays critical roles in patterning of the embryo through epithelial to mesenchymal signalling and the maintenance of stem cells in the adult organism. There is increasing evidence that this pathway is dysregulated in many malignancies, including breast cancer. While there has been a significant decrease in mortality from breast cancer, a number of treatment challenges remain, particularly in those tumours which develop resistance to endocrine-based therapy, or which lack expression of hormone or c-erbB2/HER2 receptors. Therapeutic manipulation of the Hh pathway as a potential cancer therapy is attracting great interest, with preclinical studies and clinical trials underway in a range of malignancies. This review highlights important recent developments that affect the potential of the Hh pathway as a novel therapeutic target in early breast cancer.