Cicatricial pemphigoid is a chronic, systemic, immunobullous disorder affecting mucous membranes. Nasal manifestations of cicatricial pemphigoid are less common than in the rest of the upper aero-digestive tract, and may prove difficult to diagnose and manage effectively. We report one such case presenting with isolated nasal symptoms, in which diagnosis, treatment and ongoing management of the underlying cause was particularly challenging. A literature review was performed to ascertain the incidence of cicatricial pemphigoid and to establish the best evidence-based investigation and treatment.