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Mastocytosis represents a spectrum of disease presentations. In pediatric patients, this broad category includes solitary mastocytomas, diffuse cutaneous mastocytosis, and urticaria pigmentosa [1, 2]. Presentation before puberty defines childhood mastocytosis. The majority of pediatric patients present with mastocytosis by 2 years of age. Both genders are affected equally. Mastocytosis is a disorder of mast cells leading to increased histamine release. Many pediatric cases of mastocytosis have a mutation in the c-KIT gene, which when activated promotes cell growth and survival .