Mastocytosis are characterized by an accumulation of abnormal mast cells in various tissues. Their incidence is estimated at 1/150,000 patients. Pure cutaneous mastocytosis which are mainly observed during childhood may resolve spontaneously during adolescence, whereas systemic mastocytosis involving one or more organs or tissues are more observed in adults. The initial event leading to mastocytosis is believed to be related to activating mutations in c-kit receptor, thus resulting in increased proliferation of mast cells precursors, migration in various tissues and degranulation leading to clinical signs. This nosologic entity does not belong to allergic diseases. Their perioperative management involves a multidisciplinary approach. The degranulation of mast cells with subsequent clinical symptoms can be triggered by psychological, chemical, traumatic, physical (rubbing, extreme temperatures...) agents. Avoiding these triggers should be realized whenever possible according to each patient. The premedication has not proven its efficiency. Tryptase measurement is part of the preoperative biological assessment. The clinical signs severity is related to the cardiovascular homeostasis disturbances (arterial hypotension, cardiovascular collapse, cardiac arrest). The cardiovascular symptoms do not correlate to the cutaneous versus systemic description of the disease. The drug of choice for the treatment of the severe cardiovascular signs is epinephrine associated to vascular loading. The aim of this literature review is to suggest the different modalities of perioperative care of patients with mastocytosis.