Alveolar echinococcosis is an uncommon parasitic disesae confined to the Northern Hemisphere. There is limited data regarding the incidence of the disease in Kayseri. Clinicopathologic features of the cases with the diagnosis of alveolar echinococcosis reviewed between 1980-2010. Twenty-nine cases of alveolar echinococcosis were found. There were no significant distribution differences during the study period. 28 of the 29 cases were localised in the liver, whereas one case was localised in the omentum. Sixteen of the 29 cases were male (55%) and 13 were female (45%). The age distribution of these cases varied between 33 and 80. Thirteen cases resided in Kayseri, 2 cases resided in Erzurum, 1 each case resided in Adana, Ardahan, Kars, Niǧde, Nevşehir and Yozgat. We could not obtain information from the remaining 8 cases. Abdominal pain was the main symptom in 8 cases, jaundice in 2 cases and fatigue and fever in one case on admission. One case was detected incidentally. All of the cases were diagnosed by histologic examination. The data about the alveolar echinococcosis is limited due to its low prevelance. Alveolar echinococcosis cases were detected in Kayseri with a lower incidence than in the East Anatolian region. This report will add data about the incidence of the alveolar echinococcosis.