Linear IgA disease is a subepidermal blistering disorder of unknown cause. Along with dermatologic blistering, other manifestations include oral ulceration and desquamative gingivitis. We present a case of linear IgA disease that manifested simply as oropharyngeal ulceration and desquamative gingivitis, resulting in considerable delay in diagnosis and treatment. This article (1) highlights the difficulties of diagnosing linear IgA disease when the predominant presenting features are mucosal and (2) reviews the current literature regarding linear IgA disease.