Pemphigus vulgaris (PV) is a rare, potentially fatal, immune-mediated chronic disease characterized by the presence of bullous intraepithelial lesions on mucous membranes and skin. This study aimed to perform a systematic literature review covering PV clinical and histopathological aspects and treatment. The literature searches were carried out in the Pubmed, Periódicos Capes, Scopus, Science Direct, Web of Science and Scielo databases. Articles in English or Spanish published from 2000 to 2022 comprising case reports, case series and literature reviews with case report were included. After the analyses, 21 articles were selected. PV generally presents in the third to sixth decades of life and exhibits no gender predilection. The disease manifests itself clinically through irregular and painful blisters that rupture, resulting in erosion and ulceration areas. Histopathologically, the presence of an intraepithelial cleft located above the basal layer and acantholysis are observed. Standard treatment encompasses systemic and topical corticosteroids, with prednisolone being widely employed. Management consists of a remission induction phase and a maintenance phase. An early and accurate diagnosis is paramount to quickly initiate treatment, resulting in more favorable prognoses, as the choice of treatment and responses depend on the severity of the disease. Registered at the International Prospective Register of Systematic Reviews (PROSPERO): Number CRD42024497313.