Interstitial lung diseases (ILDs) may be complicated by the development of pulmonary hypertension (PH), which is associated with worse functional impairment and a poorer prognosis. This article reviews the current state of knowledge on the prevalence, pathogenesis, diagnosis and prognosis of ILD-related PH. Whether the treatment of ILD-related PH changes clinical outcomes is currently unknown, but the current studies are summarized and the authors' perspective is offered.