Mastocytosis is a disorder characterized by a proliferation of mast cells and their subsequent accumulation in organs, such as skin, bone marrow, bone, gastrointestinal tract, liver, spleen and lymph nodes. Mast cells are derived from haematopoietic progenitors, therefore mastocytosis has to be considered an haematological disease (1, 2). Since the first description of a cutaneous form (1878) and the successive identification of systemic lesions, the aetiology remains still unknown; the treatment is substantially symptomatic, despite the studies developed for defining clinical features (1). We report on a patient admitted with a suspect of vasculitis, then recognized affected by systemic mastocytosis and herein we review the data of the literature.