There are 300 known Saussurea species. Among them, Saussurea lappa (S. lappa) is a representative perennial herb, globally distributed across Himalaya region. S. lappa has been traditionally used in medicines without obvious adverse effects. Despite significant progress in phytochemical and biological analyses of S. lappa over the past few years, inclusive and critical reviews of this plant are anachronistic or quite limited in scope. The present review aims to summarize up-to-date information on the active constituents, pharmacology, traditional uses, trade and challenges in conservation and sustainable use of S. lappa from the literature. In addition to botanical studies and records of the traditional use of S. lappa in over 43 diseases, scientific studies investigating the latent medicinal uses of this species and its constituent phytochemicals for a range of disorders are presented and discussed. The structure, bioactivity, and likely mechanisms of action of S. lappa and its phytochemicals are highlighted. Although some progress has been made, further scrupulous efforts are required to investigate the individual compounds isolated from S. lappa to validate and understand its traditional uses and develop clinical applications. The present review offers preliminary information and gives direction for further basic and clinical research into this plant.