Echinococcosis or hydatid disease is a parasitic zoonosis acquired by humans through ingestion of viable helminthic eggs of Echinococcus sp. with their food. A hydatid cyst of the spleen is a rare condition, commonly reported in 0.5-8% of patients with echinococcosis. We aim to describe herein an interesting and rare case of splenic hydatid disease diagnosed in a 34-year-old female patient residing in a rural area from Romania, a country endemic for this disorder. The therapy consisted of total splenectomy, and the postsurgical evolution was favorable.