BACKGROUND Alveolar echinococcosis (AE) is a chronic, rare and sometimes lethal parasitic infection in humans, caused by the larval stage of the fox tapeworm Echinococcus multilocularis. This study aimed to investigate the clinical aspects and treatment outcomes of patients with alveolar hydatid disease. METHODS The medical records of patients with alveolar echinococcosis admitted between 1997 and 2012 were reviewed. Diagnosis was confirmed by physical examination, ultrasonography and CT scanning and MRI. Various treatment techniques were used such as complete liver resection in seven (38.89%) patients, biliary bypass in two (11.11%) patients, laparotomy and tumor biopsy in eight (44.44%) patients and long term medical treatment in one (5.56%) patient. After discharge, all patients were followed to determine the effect of treatment, complications, recurrences and survival. RESULTS A total of 18 patients with mean age of 46.11±15.14 years (range 23-74 years) were studied. The disease was more prevalent in women than men (78.9% vs 4, 21.1%, P=0.021). Fourteen (77.78%) patients live in Chenaran, a town located in Khorasan, Iran). Death occurred in (22.22%) patients after an average period of 45.70±7.50 months after disease onset. 14 remaining patients survived after a mean follow-up duration of 54.60±29.17 months. CONCLUSIONS Diagnosis of alveolar echinococcosis should be considered in endemic area. Early diagnosis and treatment is associated with excellent outcome.