Hematopoiesis and vascular homeostasis are closely linked to each other via subsets of circulating bone marrow-derived cells with potent activity to repair endothelial injury and promote angiogenesis. As a consequence, abnormalities in hematopoiesis will eventually affect vascular health. Pulmonary arterial hypertension (PAH) is a vascular disease characterized by severe remodeling of the pulmonary artery wall. Over the past decade, circulating hematopoietic cells have been assigned an increasing role in the remodeling, such that these cells have been used in new therapeutic strategies. More recently, research has been extended to the bone marrow where these cells originate to identify abnormalities in hematopoiesis that may underlie PAH. Here, we review the current literature and identify gaps in knowledge of the myeloid effects on PAH.