We report the case of a 37-year-old man suffering from GS with recurrent episodes of angioedema, fever, hypereosinophilia [6,000/mm3 (45%)], and high eosinophil cationic protein (ECP) (>200 μg/l), treated with oral steroids during the acute phase (prednisone 50–75 mg/day), the dose of maintenance being 25 mg/day. Here, RNASE3 is linked to Gerstmann syndrome.