(1) Background: Infection with Echinococcus granulosus is the cause of hydatid cysts located frequently in the liver and lungs, but also in the kidneys, bones, spleen, brain and muscles. Cystic echinococcosis is sometimes complicated to treat, requiring radical surgery and prolonged medication. The hydatid cyst in the retroperitoneal space is frequently asymptomatic, unless it grows and produces symptoms caused by high intracystic pressure, secondary infection, wall breaking and allergy. (2) Case Report: We present the case of a 47-year old male patient, admitted for a giant bilobed retroperitoneal hydatid cyst. Following diagnosis, the patient was operated on using an anterior midline abdominal approach. The cyst was inactivated, the contents were evacuated and the cavity was drained and cleaned by opening the cyst in the middle area and at its lower pole, respectively. Subsequently, the cyst required re-installation of the drain. The evolution of the case was finally favorable but confirmed that the postoperative evolution of large hydatid cysts can be unexpected and even followed by complications. (3) Conclusions: For the presented case, the classic surgical method was the appropriate choice, considering the large size of the hydatid cyst. Bilobed cysts and those with calcified walls may cause problems with the spontaneous closing of the remaining cavity. For complicated cysts, the treatment is radical or conservative surgery, while for uncomplicated cysts, minimally invasive treatment is an appropriate choice.
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