Idiopathic pulmonary fibrosis (IPF), the most common form of chronic interstitial lung disease, is a severe progressive fibrotic disorder of unknown aetiology. The disease has a heterogeneous clinical course, with frequent poor prognosis, similar to malignant disease. Correctly diagnosing IPF has become particularly important in view of the availability of more precise therapeutic indications, thus avoiding steroid treatment and allowing new approaches with novel drugs. To date we have limited information about biomarkers predictive of progressive disease and associated complications. Efforts should be made in the future to more appropriately study lung tissue and then to extrapolate the most clinically fitting biomarkers. This approach is already used in routine management of many cancers and provides a potential road map for more appropriate clinical care of IPF. This review will mainly focus on histology and etiopathogenesis highlighting some morphological and molecular features that may influence the overall management of IPF.