Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease characterized by interstitial lung fibrosis with involvement of alveoli and terminal bronchiole. Its pathogenesis is still unknown, the risk factors involved in this disease are still unclear and its prognosis highly unfavorable. The main clinical presentations, the major and minor diagnostic criteria, the principal hypothesis on the pathogenesis of IPF and the experimental approaches for induction of the disease mostly in the murine model will be discussed together with current available treatments and ongoing clinical studies on drug therapy.