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Background and objective  Hydatid disease is endemic in sheep and cattle-raising areas world wide. Its prevalence is high in Nepal. The study was carried out to determine the clinical, radiological and pathological presentations of hydatid disease. Method This was a retrospective study of all hydatid disease cases reported in Department of Pathology, from August 1996 to July 2010. All the clinical, radiological and pathological data were collected and collated. Result    A total of 51 cases of hydatidosis were studied. Patients presented with related symptoms in 47 cases, 92.16% with CI (84.78,99.54) and asymptomatic in 4 cases, 7.84% with CI (0.46, 15.22). Involvement of liver and lung was found in 35 cases (68.63%) and 10 cases (19.61%) respectively. Involvement of other organs like kidney, pelvis and broad ligament were seen in 6 of the cases.  29 cases, 56.86% with CI ( 43.2, 70.46) had solitary cyst while rest of the cases had multiloculated cyst. All cases had radiological correlation and histopathological confirmation.  Conclusion Most cases presented with organ related vague symptoms, however it should be considered as a differential diagnosis especially in asymptomatic cases and cases with unusual sites. Imaging studies is useful in preoperative diagnosis and postoperative histopathology is confirmatory. A multi centric hospital based study will help to decrease the incidience.