Background: Hydatid disease (HD) is still an important health hazard in the world. This disease is a parasitic infestation endemic in many sheep- and cattle-raising areas such as Iran. Objectives: This study aimed to review the clinical manifestations, laboratory aspects, imaging findings, and management of HD. Patients and Methods: Data were collected from the medical records of patients diagnosed with HD in eight referral hospitals in different provinces of Iran from 2001 to 2014. Results: Overall, 161 children at a mean age of 9.25 ± 3.37 years (age range = 1 - 15 years old) hospitalized with a definite diagnosis of the hydatid cyst between 2001 and 2014 were studied. The male-to-female ratio was 1.6:1. The most commonly involved organ was the lung (67.1%), followed by the liver (44.1%) and a combined liver and lung involvement was found in 15.5% of the patients. The cysts were found more frequently in the right lobe of the liver and lung than in the left lobe. The most frequent complaints were fever (35.4%) and abdominal pain (31.7%), and the most frequent sign was an abdominal mass in the liver involvement and cough in the lung involvement. There was a high eosinophil count (> 500/micL) in 41% of our cases. A high erythrocyte sedimentation rate (> 30) or positive C-reactive protein (based on the qualitative method) was found in 18.6% of the patients and leukocytosis > 15000/micL in 29.2% of the children. Ultrasonography was the main imaging test, with an accuracy rate of 96%, and chest X-ray was helpful in 88.6% of the cases. Surgery was performed in 89% of the patients, and selective patients underwent percutaneous aspiration-injection-reaspiration drainage or medical treatment. Conclusions: The lung was the most commonly involved organ in the children recruited in the present study. Given the high probability of multiple organ involvement, we recommend that patients with HD be assessed via ultrasonography and chest X-ray. In endemic regions, unexplained eosinophilia should be considered as a parasitic disease like HD and its complications.