Alveolar echinococcosis (AE) is a chronic zoonotic parasitic disease caused by infection with Echinococcus multilocularis . AE is associated with a high mortality rate and poses a significant threat to human health. The primary treatment for AE is surgical resection of the lesions; however, owing to its long incubation period and insidious disease progression, many patients are diagnosed only after the onset of complications such as liver cirrhosis, jaundice, and portal hypertension, which preclude curative surgical intervention. For patients who are unwilling or unable to undergo surgery, lifelong administration of anti-AE medications is necessary. Benzimidazole compounds, such as albendazole and mebendazole, are the current mainstays of treatment, offering good efficacy. Nevertheless, these medications primarily inhibit parasite proliferation rather than eradicate the infection, and their long-term use can lead to significant drug-related toxic effects. Consequently, there is an urgent need to develop new therapeutic strategies that convey better efficacy and reduce the adverse effects associated with current treatments. Recent advancements in AE therapy include novel synthetic compounds such as antiviral agents, antibiotics, antineoplastic agents, immunosuppressants, and antiangiogenic agents, as well as natural compounds derived from traditional Chinese and Tibetan medicine. These new drugs show promising clinical potential because they interfere with parasitic metabolic pathways and cellular structures. This review aims to discuss recent research on AE drug therapy, including mechanisms of action, dosing regimens, signalling pathways, and therapeutic outcomes, with a goal of providing new insights and directions for the development of anti-AE drugs and summarizing current advancements in AE pharmacotherapy.