The grounds for rare involvement of conjunctiva in pemphigus vulgaris is unclear. We hypothesize that inactivation of conjunctival desmoglein 3 may be compensated by other desmosomal proteins. Severe conjunctivitis may be the dominating clinical manifestation in pemphigus vulgaris. This implies a need of establishing distinct severity criteria and therapeutic standards for ocular pemphigus. In our patient rapid clinical response was achieved after introducing combined treatment with prednisone and oral cyclophosphamide.