Pulmonary hypertension (PH) may complicate the course of many forms of advanced interstitial lung disease (ILD) and has been shown to portend a worse outcome. The aetiology of PH is likely multifactorial with variable contribution of factors amongst the different diseases. The most common such conditions include idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-related ILD and pulmonary langerhans cell histiocytosis. Whether the course and impact of PH in these conditions can be modified by therapy requires further study.