The Himalayan region of India, known for its rich biodiversity, includes numerous medicinal plants such as Aconitum balfourii Stapf. This review critically examines A. balfourii, focusing on its classification, distribution, medicinal significance, and the pressing need for conservation. A comprehensive literature review was conducted using databases like PubMed, Google Scholar, and Web of Science. Studies on the taxonomy, geographical distribution, medicinal applications, and conservation status of A. balfourii were included, incorporating both in vitro and in vivo research, as well as ethnobotanical and conservation studies. Predominantly found in Uttarakhand, A. balfourii contains key alkaloids such as pseudoaconitine, aconitine, picroaconitine, haemonepellene, and benzylaconitine. These compounds exhibit diverse pharmacological activities, including antioxidant, anti-inflammatory, analgesic, cardiotonic, and vermifuge properties. Despite its medicinal potential, A. balfourii is declining due to overexploitation, climate change, and insufficient regeneration rates. Traditionally, A. balfourii has been used to treat fever, swelling, sciatica, rheumatic pain, neuralgia, and other ailments. Recent research has identified new norditerpenoid alkaloids, expanding their pharmacological profile. Studies on the plant’s adaptability to different altitudes and elevated CO2 levels highlight its growth and secondary metabolite production. Micropropagation techniques and efficient DNA isolation protocols have been developed to preserve its genetic diversity. This review underscores the necessity for both in-situ and ex-situ conservation strategies for A. balfourii. Coordinated efforts among governmental bodies, research institutions, and local communities are vital for its sustainable use and long-term preservation. Enhancing awareness of its medicinal value and ecological significance is essential to ensure its conservation, thereby supporting the biodiversity of the Himalayan region and traditional medicinal practices. © The Author(s) under exclusive licence to Society for Plant Research 2024.