Pulmonary hypertension (PH) is a highly prevalent and important condition in adults with chronic kidney disease (CKD). In this review, we summarize the definition of PH, discuss its pathophysiology and classifications, and describe diagnostic and management strategies in patients with CKD, including those with kidney failure treated by kidney replacement therapy. In the general population, PH is classified into 5 groups based on clinical presentation, pathology, hemodynamics, and management strategies.