Introduction and objective: Mastocytosis is a rare, heterogeneous group of disorders characterized by clonal proliferation and accumulation of mast cells in various organs, most commonly the skin and bone marrow. The aim of the review is to summarize current knowledge on epidemiology, pathogenesis, diagnostic criteria and therapeutic strategies in mastocytosis, with special emphasis on novel targeted treatments. Review methods: A structured literature search was performed using PubMed, Scopus, and Web of Science. Articles published between January 2015 – October 2025 were included. This period was chosen to reflect recent advances in the understanding and treatment of mastocytosis, including developments in molecular diagnostics and targeted therapies. Relevant publications were selected based on their contribution to the topic, with emphasis on clinical relevance. Priority was given to clinical trials, reviews, and guidelines. Brief description of the state of knowledge: The pathogenesis of mastocytosis is dominated by activating KIT mutations, primarily D816V, which stimulate mast cell proliferation and survival through JAK-STAT, MAPK and PI3K pathways. Additional mutations including TET2, ASXL1, SRSF2 and RUNX1 contribute to more aggressive disease and worse prognosis. Symptomatic therapy remains the foundation of management for most patients and includes H1/H2 antihistamines, leukotriene antagonists, proton pump inhibitors and mast cell stabilizers. Summary: Advances in the understanding of molecular basis, improved diagnostic precision and the development of potent selective KIT inhibitors have transformed the management of mastocytosis. Symptomatic therapy remains essential for indolent forms, while targeted agents provide effective options for severe variants. Nevertheless, there is a need to further develop research in this area.