Introduction. Hydatid disease is caused by the tapeworm Echinococcus granulosus and is still a matter of public health in many regions of the world, where it is an endemic parasitic disease. Although the liver is the most involved organ, hydatidosis can be found anywhere in the human body. Rare forms of location may lead to diagnostic and therapeutic dilemmas. Case Report. Herein we report a rare case of acute abdominal pain and progressively increasing abdominal distension due to abdominal and multiple splenic echinococcosis in a 72-year-old Caucasian male. We also provide a brief review of the literature. Conclusion. Although hydatid disease is found most often in the liver and lungs, rarely any organ of the body can be involved by this zoonosis. Though rare, the possibility of unusual location of echinococcosis must always be considered by the operating surgeon, when dealing with diffuse abdominal pain in endemic areas, because any misinterpretation may result in unfavorable outcomes.