We report a new case of hyper-IgD syndrome, a recently described disease characterized by recurrent episodes of fever with headache, bilateral cervical lymphadenopathy and, more rarely, abdominal pain and diarrhoea. Polyclonal increase of serum IgD is the most important laboratory finding. Etiopathogenesis and differences with familial Mediterranean fever are discussed. Moreover, good results obtained with colchicine treatment are also reported.