Paraneoplastic pemphigus is a rare condition with extremely high rates of mortality. Although its pathogenesis is incompletely understood, its pathologic findings have significant overlap with other autoimmune blistering diseases, such as pemphigus vulgaris, bullous pemphigoid, erythema multiforme and erosive lichen planus. A universally accepted consensus definition is needed to firmly define the condition. This would aid in identification of paraneoplastic pemphigus and the institution of timely and appropriate treatment to avoid rapid patient deterioration as well as recruitment for trials to further examine the pathogenesis and new therapeutic modalities. This article reviews the varied clinical presentations and pathologic characteristics pertaining to paraneoplastic pemphigus.