Hydatid disease, which is caused by Echinococcus granulosus, is a common public health problem worldwide. The hydatid cysts may take many years to produce clinical symptoms. The liver is the organ most commonly affected. Diagnosis is made by a combination of clinical, imaging, serological and molecular techniques. Primary axillary hydatid disease is extremely rare and only 9 cases have been reported up to now. Herein, we reported a 48-yearold case with primary isolated hydatid disease in the axilla in whom total cystectomy was performed.