Although the etiology of such dysfunction cannot be fully understood, other case has already been reported in the literature consisting of an adult onset Fanconi syndrome with kidney medullary cystic disease, non-specific aminoaciduria, lysozymuria and beta2-microglobulinuria, hyperreninemia and polycythemia with elevated serum EPO levels resembling Bartter’s syndrome.(13) Another characteristic phenotype usually described in BS cases is the hypercalciuria, associated or not to nephrocalcinosis. Here, EPO is linked to polycythemia.