Langerhans cells (LCs) were first described by Paul Langerhans, in 1868, as dendritically shaped cells, which were located in the squamous epithelia of epidermis. Later on, these cells were identified in all stratified squamous epithelium of mammals. Dendritic cells (DCs) play an important role in local defense mechanisms in the epithelium. LCs are situated usually in the suprabasal layer of stratified squamous epithelia of oral mucosa and epidermis of skin. They constitute 3% of the cell population in epidermis. LCs are thought to act as antigen presenting cells (APCs) during initiation of immune responses. With the help of APCs, the lymphocytes are able to recognize and respond to specific microbes. In this paper we have reviewed the origin, distribution, demonstration and mechanism of action of LCs and their role in different pathological conditions.