Hydatid disease (HD) is a zoonotic infection caused by larval stage of Echinococcus granulosus. It is a worldwide health problem causing significant socioeconomic impact. This retrospective study included 19 patients diagnosed with HD at a university medical center between 2007 and 2019 in Western Region of Saudi Arabia. There were 68% (n=13) males and 32% (n=6) females (mean age 40 ± 17 years). The clinical presentations were variable and non-specific. The affected organs were as follows: liver (68%), lung (32%), soft tissue (11%), peritoneal nodule (5%), kidney (5%), and brain (5%). Multiple organ involvement and combined liver and lung were seen in 26% (n=5) and 16% (n=3) of the patients, respectively. The mean diameter of cyst was 8.98 ± 1.77 cm. Serology confirmatory test was positive in 95% (n=18) of the patients. Blood eosinophilia was noticed in 32% (n=6) of the patients. Histopathological and cytological features include laminated cyst wall, inner germinal layer and protoscolices. Management included cyst resection or PAIR (Puncture, Aspiration, Injection of protoscolicidal agent and Re-aspiration) technique combined with anthelmintic drugs. The overall recurrence rate was 7.5% and 60% for surgery and PAIR, respectively. Overall mortality rate was 0%. In conclusion, considering that there is no pathognomonic clinical feature for HD, it may lead to confusion and delaying in diagnosis. Good history including exposure to dogs in the endemic areas, good imaging modalities and the proper use of serology might help for suspecting HD. The histopathological findings are valuable in confirming diagnosis of HD.