A case of immunotactoid glomerulopathy in an 18-year-old man with an idiopathic hypereosinophilic syndrome is presented. The patient showed cervical lymphadenopathy, asymptomatic proteinuria of nephrotic range, and hematuria without any defined immunologic disease. Marked and prolonged hypereosinophilia was found in peripheral blood (eosinophil count; 6,248/mm3) and bone marrow (eosinophil series; 32%). Diffuse and/or nodular eosinophilic infiltration was identified in multiple organs such as kidney, stomach, liver, lymph node, and skin. Renal biopsy revealed endocapillary proliferative features of typical immunotactoid glomerulopathy with IgG and C3 deposition and microtubular structures of variable size, 20-80 nm in diameter, mainly in the subendothelium. This study suggests that immunotactoid glomerulopathy may be a secondary immunologic manifestation of the tissue damage by eosinophils in the idiopathic hypereosinophilic syndrome.