Pemphigus vulgaris (PV) is an autoimmune blistering disorder with a complex etiology involving an interplay of genetic as well as environmental factors, most of which remain unknown. Despite the identification of several human leukocyte antigen (HLA) alleles as risk factors for disease, no other non-HLA genes have clearly been implicated in disease susceptibility. Newer candidate gene and whole-genome approaches are needed to illuminate the full palate of genetic risk elements in PV. Based on this information, genetic-based tools can be expected to provide a scientific rationale for future clinical decision-making by physicians and facilitate an era of personalized medicine.