Livedoid vasculopathy (LV) is characterized by painful purple macules and papules that subsequently ulcerate. The lesions heal over weeks to months resulting in smooth, porcelain-white, atrophic plaque-like areas with surrounding telangiectases and hyperpigmentation. The specific cause of LV is still to be determined and it is believed to be multifactorial in nature. Despite numerous available therapeutic agents, there is not a single best efficacious treatment for LV. Most of the available treatment options are based on isolated case reports or case series. In this article, studies on the pathogenesis and therapeutic approaches to LV are reviewed.