OBJECTIVE To examine recent advances in the understanding of the prevalence, pathophysiology, natural history, diagnosis, and treatment of pulmonary hypertension associated with interstitial lung disease. RESULTS Significant progress has recently been made in understanding the etiology of various causes of pulmonary hypertension, including those associated with interstitial lung disease, and new data regarding the various pathophysiologic mechanisms of pulmonary hypertension in interstitial lung disease have been published. Therapeutic agents that were initially studied in patients with idiopathic pulmonary arterial hypertension are now being applied to interstitial lung disease-related pulmonary hypertension. Short-term hemodynamic benefits and disease-related quality of life outcomes that are associated with these advanced therapies are now better understood, but data regarding long-term outcomes and the efficacy of combination therapies remain lacking for interstitial lung disease-related pulmonary hypertension. Lung transplantation outcomes in the treatment of pulmonary hypertension associated with interstitial lung disease have been further defined. CONCLUSIONS Pulmonary hypertension associated with interstitial lung disease is now frequently recognized and is associated with substantial morbidity and mortality. Various new pharmacotherapies as well as lung transplantation are increasing the treatment options for these disorders, although timing of advanced therapies and long-term benefits and toxicities remain uncertain.