Hydatidosis is an endemic parasitic disease in Mediterranean region, Middle east, Australia, parts of Africa, Latin America and Turkey. The cysts are mostly evident in the liver or lungs, while urinary tract involvement is uncommon, comprising only 2-4 % of all cases. Isolated renal involvement is extremely rare. Its diagnosis may be difficult because of nonspecific complaints and absence of pathognomonic laboratory findings except from hydatiduria. Although radiological studies have a more important role in the diagnosis, they cannot always show a specific sign or lesion for hydatid disease. Herein we present a rare case with isolated renal hydatid cyst mimicking a renal mass treated with right radical nephrectomy. Isolated renal cyst hydatid should be considered in differential diagnosis of both cystic and solid renal masses.