Mastcytosis is a rare disease characterized by an abnormal increase of mast cells in tissues. The skin is the organ most frequently involved, but mast cells also accumulate in the bone marrow, gastrointestinal tract, lymph nodes, spleen, and liver. Recent studies suggest that activating mutations of c-kit, a protooncogene encoding for the receptor (kit) of stem cell factor, are a possible cause of some forms of mastocytosis. In addition, an increased rate of chromosomal aberrations has been found. Despite significant advances in research on mastocytosis, curative treatment is not yet available. Current management is based on avoidance of mediator-releasing triggers and symptomatic treatment.