Objective: To describe the epidemiology, etiology, pathogenesis, clinical characteristics, and management of pemphigus, with an emphasis on ocular involvement. Methods: Literature review. Results: Pemphigus is an autoimmune epithelial blistering disease of the skin and mucous membranes. The typical pathological finding is acantholysis of the epidermis that leads to blister formation. Immunofluorescence techniques show autoantibody deposition on the epidermal intercellular substance. Although a genetic background is necessary, environmental factors are crucial for the onset and perpetuation of the disease. Exposure to some drugs, toxic agents, and foods and associations with other autoimmune diseases and lymphoproliferative conditions should be assessed. Generally, the skin is the most commonly affected tissue. Ocular involvement might be present and exhibit a clinical course that is independent of skin compromise. Visual function may be affected depending on the severity of the presentation. In untreated cases, mortality is high because of bacterial sepsis and hydroelectrolyte imbalance. A multidisciplinary approach should be used involving a dermatologist, ophthalmologist, and immunologist. Immunosuppressive agents are the mainstay of treatment; corticosteroids typically with azathioprine or mycophenolate mofetil are the drugs of choice. Surgical treatment of trichiasis and malposition of the eyelids and tectonic procedures for corneal perforation are sometimes required in very severe and recalcitrant cases. Conclusions: Pemphigus is a potential life-and sight-threatening disease. Understanding the disease facilitates the adequate assessment of the modifiable factors and the prompt initiation of immunotherapy. Ocular involvement can develop in patients with pemphigus. Adequate ophthalmological care is needed, in particular, prevention of infections, scarring, and corneal perforation.