Langerhans cell histiocytosis (LCH) is an intricate and misunderstood disorder marked by the clonal proliferation of bone-marrow derived Langerhans cells along with eosinophils, macrophages, lymphocytes and sometimes, multinucleated giant cells. This is a literature review that discusses the gradual change and development in diagnosis and treatment of cutaneous LCH in adults. In spite of the complexity of the disease and usually being a multi-systemic disorder involving various organs, involvement of skin is very rare in adults. Since there are few cases that have been described, researched and reviewed by the experts in this field we select it to put a light on the matter. We choose a qualitative review instead of quantitative because of the limited documents available and fewer cases in skin-limited adult disease but the accurate diagnosis of the skin lesions is important to carry out the required treatment, avoid undiagnosed or misdiagnosed cases and improve prognosis. This manuscript includes articles from pubmed, google scholar and medline in English language indexed with keywords "Langerhans cell histiocytosis, skin-limited, adult, diagnosis, treatment". Recent identification ofBRAF-V600E has helped in specific diagnosis and target treatment of the disease.