Echinococcosis or hydatid disease is a near-cosmopolitan parasitic zoonosis caused by the larval stages of cestodes of the genus Echinococcus, whose spectrum of clinical manifestations can vary from asymptomatic infection to death, the liver being the most frequently affected organ. In the absence of an adequate treatment, case fatality rate of affected patients may reach 100%, so clinical suspicion should always be accompanied by a complete workup and a prompt treatment. Like other zoonotic parasitic diseases, echinococcosis is associated with low socioeconomic and cultural levels, especially affecting rural areas of developing countries, not only by the morbidity and mortality it produces, but also by the economic impact associated to health care costs and productivity losses. Despite this, in Colombia and in other South American countries, epidemiological studies of this disease are limited, so it is not known with certainty the incidence, the prevalence, and the burden of the disease. The objective of this chapter is to summarize and describe epidemiological and clinical updated aspects of the disease, focusing on evidences and published research articles on echinococcosis in Colombia, finally questioning if this cestode zoonotic disease has been largely neglected.