Background: Hydatid cyst disease (HCD) or echinococcosis has a worldwide distribution and is endemic in many countries of Mediterranean region. In Iran, due to various climatic conditions, the rate of the disease is diverse in different parts. The most infected regions are the domains of Alborz and Zagros Mountains where there is a high rate of ruminants and the main carrier of people are animal husbandry. Due to the importance of this zoonotic helminth and the lack of information about hydatid cyst disease in Guilan province, we conducted a retrospective study from 2000 to 2010 to evaluate the HCD inpatients in this region. Objectives: The aims of the study are to evaluate HCD inpatients and to review the clinical presentations of different organs affected by cysts and postoperative outcomes of hydatid cyst in Guilan province. Materials and Methods: A retrospective descriptive investigation was performed by reviewing the medical records of patients with HCD, admitted to four private and governmental hospitals, in Rasht, capital of Guilan province from 2000 -2010. Results: Sixty-two patients (38 males, 24 females) with a mean age of 46.38 ± 18.36 years (aged between 8 and 81 years old), with HCD were evaluated. In the 40 -60 years old group, there was highest rate of HCD (40.3%). Housewives had the highest rate of infection (40.3%) followed by farmers (22.6%), pupil (8.1%), animal husbandries (6.5%) and others (22.6%). The most prevalent complaint of the patients with liver involvement were abdominal pain (75%) and nausea and vomiting (9.1%) and the most common findings of the inpatients with lung involvement the most common complaint was cough (40%). Seventy one percent of the patients had liver involvement and 16.1% had lung involvement. The most common diagnostic method was ultrasonography (48.14%). Computed tomography was diagnostic in 43.20% of cases and chest X-ray in 8.6% of cases. Overall, 96.8% of patients were cured and the mortality rate was 3.2%. Conclusions: Echinococcosis is still an important health problem in Iran that needs further studies. Therefore, accurate information on the distribution of the disease is the first step to control and prevent it. In general, the situation of the hydatidosis in the human in Guilan shows somehow a resemblance with the other areas in Iran.