Hydatid disease may affect several organs in the human body and thus represents a major challenge for the general surgeon. The aim of this study was to analyze the multiple clinical presentations of hydatid disease and the surgical options according to the involved organ. The medical records of 272 adult patients operated on for hydatid disease in our department during the period 1970-1996 were retrospectively reviewed. The most commonly involved organ was the liver (204 patients; 75%), followed by the lung (42 patients; 15.4%) and the spleen (14 patients; 5.1%). In 12 patients, the cysts were located in uncommon sites: in the pancreas (4 patients; 1.5%), the gallbladder (2 patients; 0.74%), the kidney, the thyroid gland, the breast, the pericardium, the supraclavicular region and the thigh (1 patient in each case; 0.37%). Clinical symptomatology varied according to anatomic location and pre-operative diagnosis was accomplished with plain abdominal films, ultrasound, computerized tomography and serological tests. The surgical procedures performed included simple closure with drainage, unroofing of the cyst with omentoplasty (for liver cysts), marsupialization, cyst excision, excision of the involved organ or combinations of procedures. Postoperative morbidity was mainly due to septic complications (n = 41), postoperative bleeding (n = 2), deep vein thrombosis (n = 2) and fistulae (n = 13). Four patients died in the early postoperative period (mortality rate, 1.5%) secondary to septic complications (n = 3) and pulmonary embolism (n = 1). During long-term follow-up, 14 patients developed recurrent disease. In conclusion, hydatid disease should be included in the differential diagnosis of cystic masses in solid organs or other anatomic sites, especially in endemic countries. Since there is not an effective medical treatment, surgery still remains the treatment of choice, offering a good clinical result and an acceptable recurrence rate.