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Minor diagnostic criteria include elevated serum tryptase level, abnormal MC CD25 expression, and presence of <jats:italic>KIT<\/jats:italic>D816V mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk stratification<\/jats:title><jats:p>Establishing SM subtype as per the World Health Organization classification system is an important first step. Broadly, patients either have indolent\/smoldering SM (ISM\/SSM) or advanced SM, the latter includes aggressive SM (ASM), SM with associated hematological neoplasm (SM\u2010AHN), and mast cell leukemia (MCL). Identification of poor\u2010risk mutations (ie, <jats:italic>ASXL1, RUNX1, SRSF2, NRAS<\/jats:italic>) further refines the risk stratification. Recently, clinical and hybrid clinical\u2010molecular risk models have been developed to more accurately assign prognosis in SM patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Management<\/jats:title><jats:p>ISM patients have a normal life expectancy and treatment is generally limited to anaphylaxis prevention\/symptom control\/osteoporosis treatment. Patients with advanced SM frequently need MC cytoreductive therapy to ameliorate disease\u2010related organ dysfunction. High response rates have been seen with small\u2010molecule inhibitors that target mutant\u2010KIT, including midostaurin (Food and Drug Administration approved) or avapritinib (investigational). Other options for MC cytoreduction include cladribine or interferon\u2010\u03b1, although head\u2010to\u2010head comparisons are lacking. Treatment of SM\u2010AHN primarily targets the AHN component, if an aggressive disease such as acute myeloid leukemia is present. Allogeneic stem cell transplant can be considered in such patients, or in those with relapsed\/refractory advanced SM. 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