Cystic echinococcosis is a zoonotic infection of humans and domestic animals caused by the larval stages of the cestode Echinococcus granulosus. Cystic echinococcosis is one of the most important zoonotic diseases in Iran, where human cases are frequently reported from different regions of the country. The objective of this study was to determine the sero-epidemiological and surgical cases of human hydatidosis in the Yasuj district, a region in southwest of Iran. Serum samples were taken from 500 individuals attending Yasuj health centers. Anti-hydatid cyst antibody was checked by enzyme-linked immunosorbent assay (ELISA), using antigen B. Moreover, retrospective studies were carried out using local hospital records of CE patients between 1997 and 2006. Results of the sero-epidemiological study showed that 36 out of 500 cases (7.2%) have antibody against hydatid cyst. Of these 36 cases, 49.6% were women and 50.4% were men. The highest rate of infection was recorded in individuals aged between 30 and 39 years. Hospital records showed that during the 10 years, 105 cases of hydatidosis were admitted in Yasuj hospitals. Of all cases, 70 (66.7%) were women and 35 (33.3%) were men. Hepatic cyst was recorded in 81% of the cases where nephrotic cysts were the second most prevalent ones. Recurrence of the disease was noted in 14.3% of all cases. Results of this study demonstrated that hydatidosis is an important endemic disease, with a nearly constant prevalence rate during the last 10 years, in Yasuj district in Iran. Further studies are needed to investigate the reasons for high rate of surgical cases in housewives and striking prevalence of hydatidosis in a specific (30–40 years) age group.