The records of 100 children with hydatid disease were reviewed retrospectively from 1978 to 1997; 43 were girls and 57 were boys. The mean age was 9.14 years; 61 patients had 124 hepatic cysts. Presenting symptoms were asymptomatic abdominal masses, found masses incidentally during ultrasonography (US), or acute abdomen. Plain X-ray films, US, or computerized tomography (CT) are sufficient for diagnostic evaluation in endemic areas. In the differential diagnosis, laboratory investigations such as the Casoni and Weinberg tests, indirect hemagglutination, eosinophilia, and ELISA were also used. These tests may give negative results, however, in some patients with hydatid disease. The mean follow-up time was 10.5 years (range 1-18 years), the mean duration of hospitalization 7 days. The complication rate was 3.6%. Mortality was 3.27% and occurred after the administration of formaldehyde and hypertonic scolicidal agents. Hydatid disease of the liver can be treated medically in selected patients; conservative surgical approaches that save as much parenchyma as possible, such as partial cystectomy and capitonnage, are indicated in the other cases.