Idiopathic pulmonary fibrosis (IPF) is an increasingly common condition which poses many challenges. High resolution CT scanning offers a non-invasive technique which can secure an accurate diagnosis in the majority of patients. Lung function estimates of gas transfer allow the definition of advanced (DLco < 39% predicted) and limited disease (DLco > 40% predicted).Developments in the area of medical therapy have been slow. Based on current thinking regarding the pathophysiology of IPF, steroids are increasingly seen as having only a limited role in its treatment. Emerging studies are highlighting the importance of alternative forms of therapy including N-acetylcysteine, pirfenidone and bosentan, for example. Lung transplantation is a legitimate surgical treatment for IPF. However, in light of the high death rate on the waiting list, it is recommended that selected patients are referred early to the transplant programme.