Gerstmann’s syndrome in patients with hydatid cyst has not been reported frequently, although many cerebral hydatid diseases have been detailed. Tsukamoto et al. reported such a case in a patient who had a cysticercosis in his left angular gyrus. MRI showed a focal lesion situated subcortically in the left angular gyrus and reaching the superior posterior parietal lobe in a patient with Gerstmann’s syndrome. Four symptoms of Gerstmann’s syndrome are seen in left angular gyrus lesions in right-handed persons. Brain involvement is seen in 1 to 2% of all echinococcus granulosus infestations and the primary form is very rare. The diagnosis is established with serologic and imaging techniques. A round, cystic lesion is detected on the CT in cerebral hydatid disease. Cerebral hydatid cysts should be removed without rupture. However, only half of these cysts are removed unruptured. For this reason, all patients have to receive mebendazole therapy. Cases with a hydatid cyst first representing Gerstmann’s syndrome have rarely been reported. We are reporting this case due to its rarity.