Mastocytosis most frequently manifests in the skin, so most often is diagnosed by dermatologists at first. Low threshold in clinical suspicion helps early detection of these cases. Cutaneous mastocytosis is an increase of cutaneous mast cells without known underlying disease or inflammatory infiltrate. A skin biopsy with staining specific for mast cells (toluidine blue, Wright-Giemsa) and tryptase immunohistochemical analysis should be considered in all patients with characteristic skin lesions. There is no treatment that cures cutaneous matocytosis and the major concern in the treatment is patient's quality of life. Although systemic mastocytosis occurs in greater than 25% of cases of cutaneous matocytosis in adults, there is little evidence that symptomatic therapy substantially alters the course of the disease. Almost all patients with cutaneous matocytosis belong to the indolent category of the consensus revised classification and have a good prognosis. This review aimed to provide best practices recommendations based on the evidence and patients perspectives.