The lymphatic vasculature comprises an intricate network of vessels critical for fluid homeostasis, immune surveillance and fat absorption. Recent studies have provided insights into the developmental processes and molecular mechanisms controlling the formation and remodelling of the lymphatic vessels. These studies have further demonstrated the essential and active role of the lymphatic vessels in various pathological conditions and advanced our understanding of the progression of human diseases, such as inflammation and tumorigenesis. In the context of the latest exciting findings, we review here the current understanding of the mechanisms of lymphatic development and contribution of lymphatic vessels to pathological conditions. BioEssays 29:1192–1202, 2007. © 2007 Wiley Periodicals, Inc.