Hydatid disease results from infection with the larval forms (metacestodes) of Echinococcus species. However, due to its rarity and geographic distribution, it remains a neglected zoonosis that has a global impact. The primary etiological agents include E. granulosus, E. multilocularis, E. vogeli, and E. oligarthrus, each associated with distinct clinical syndromes—namely cystic, alveolar, and polycystic echinococcosis. Transmission occurs via the fecal-oral route, typically from definitive hosts such as dogs and wild carnivores to intermediate hosts like livestock, and occasionally to humans. The complex life cycle of the parasite involves environmental contamination and subsequent ingestion of eggs that result in hydatid cysts in target organs, most commonly the liver and lungs. Global burden data reveal a substantial socioeconomic impact, particularly in pastoral communities across Eurasia, Africa, South America, and the Middle East. With estimates indicating nearly 900,000 disability-adjusted life years (DALYs) and billions of dollars in annual losses, hydatid disease remains a critical public health concern. Preventive strategies include regular deworming of dogs, regulated slaughter practices with appropriate disposal of the carcasses, and livestock vaccination. Despite successful eradication in countries like New Zealand and Cyprus, the disease persists in endemic regions due to challenges in enforcement, public education, and veterinary services. Diagnosis is based on imaging, serologic testing, and clinical suspicion, while treatment options include medical therapy (e.g., albendazole), percutaneous aspiration (PAIR), or surgery, depending on cyst characteristics. A One Health approach—integrating veterinary, human, and environmental health interventions—is essential for long-term disease control. The chapter highlights the need for global collaboration, vaccine development, and strengthened surveillance to reduce transmission and improve outcomes by reviewing the current information in the literature.