PURPOSE OF REVIEW: Pulmonary hypertension is a common and devastating complication of chronic kidney disease (CKD). Traditionally considered a consequence of volume overload, recent findings now expand this paradigm. These novel mechanisms herald new treatment options. This review summarizes the current evidence to provide a theoretical model of the contributing factors for CKD-associated pulmonary hypertension. Along this framework, we highlight current and emerging therapeutic strategies for each putative factor. RECENT FINDINGS: A series of retrospective studies of right heart catheterization data provide insights into the potential hemodynamic profile of CKD-associated pulmonary hypertension. These studies suggest that elevated pulmonary vascular resistance may commonly contribute to pulmonary hypertension. In addition, preclinical models implicate an increasing array of CKD-associated factors which influence pulmonary vascular biology. Many of these factors also adversely affect kidney function and CKD progression. Clinical trial and other prospective data for treatments of CKD-associated pulmonary hypertension remain limited. SUMMARY: Volume overload and left-ventricular dysfunction are the predominant focus of CKD-associated pulmonary hypertension treatment for most patients. However, new findings suggest that treatments targeting pulmonary vascular vasoconstriction and remodeling may be promising treatment options for select patients. Clinical trials are needed for all therapeutic strategies for CKD-associated pulmonary hypertension.