Pulmonary hypertension (PH) commonly complicates the course of interstitial lung disease (ILD). Development of ILD-PH has significant implications, as it is associated with morbidity in the form of increased dyspnea on exertion and need for supplemental oxygen, as well as attenuated survival. The incidence of ILD-PH increases over time, with as many as 80% of patients with end-stage idiopathic pulmonary fibrosis developing PH. Even modest elevations in pulmonary artery pressures are associated with decreased survival. Given the prognostic significance of even mild elevations in pulmonary pressures, a high index of suspicion is required to make the diagnosis. Numerous clinical, laboratory, radiographic, or pulmonary function parameters may be suggestive of the development of ILD-PH including elevated brain natriuretic peptide levels, decreased six-minute walk test distance, increasing exertional desaturation, increased pulmonary artery to aorta ratio, and decreased diffusing capacity of the lung for carbon monoxide (DLCO). No single parameter by itself is diagnostic of ILD-PH and attempts to combine parameters into a predictive diagnostic formula have been largely unsuccessful. Even transthoracic echocardiography (TTE) lacks sensitivity for the diagnosis given difficulties in visualization of the right ventricle (RV) in ILD and minimal structural changes of the RV observed with the mild, yet important elevations in pulmonary artery pressure seen in ILD-PH. Right heart catheterization (RHC) remains the only reliable means of making the diagnosis of ILD-PH. Treatment of ILD-PH remains somewhat controversial. There is fairly universal consensus on general measures such as providing appropriate treatment of the underlying lung disease, provision of supplemental oxygen when required, diuretics for management of volume overload, and treatment of sleep disordered breathing. When to use pulmonary hypertension medications is more controversial. In 2021, the first and only randomized, controlled trial to demonstrate therapeutic efficacy of pulmonary vasodilator therapy led to the approval of inhaled treprostinil for treatment of ILD-PH. 2022 guidelines from the European Society of Cardiology/European Respiratory Society recommend restricting treatment with inhaled treprostinil to only those with severe ILD-PH, although the trial did not restrict enrollment to this population. However, the Taskforce on PH in Chronic Lung Diseases from the 7th World Symposium on PH (WSPH) recommended phenotype-based approach to patient selection including those with PVR ≥ 4. Both guidelines also make a recommendation for use of sildenafil in ILD-PH based primarily on real-world and observational data. Further study is required to improve our understanding of ILD-PH and better delineate optimal treatment strategies.