Cystic echinococcosis (CE) remains an important health problem in many areas of the world, including the Mediterranean region. We performed a retrospective study of cases reported from 1998 to 2012 in order to review and update the epidemiology of this disease in a highly endemic area situated in western Spain. A total of 471 patients were diagnosed with hydatid disease. Of these cases, 55·8% were male, with an average age of 62·3 ± 19·5 years. More importantly, 1·5% of patients were children, and 20·5% were aged <45 years. An active therapeutic approach was implemented for 92·6% of the CE patients with primary diagnoses; however, a ‘watch and wait’ strategy was used in 59·3% of all secondary CE diagnoses. The incidence rate of hydatid disease was significantly higher compared to the incidence described in the Notifiable Disease System in this area. Furthermore, a significant decrease in hydatid incidence during the years included in the study was observed (β = −0·4357, P < 0·001). CE incidence has diminished in recent years, although active transmission remains in paediatric cases. Additionally, CE incidence remains high in our region despite public health plans for its control. The documented incidence of CE disease clearly underestimates the real numbers.