Background: Pemphigus vulgaris is an autoimmune bullous disease characterized by blistering and erosions within skin and mucous membranes. Lesions appear most commonly on mucosal surfaces of the oral cavity. Ocular involvement in patients with PV has rarely been reported. Main observation: A 47-year-old male patient with a 2 month history of oral erosions and dysphagia developed severe conjunctivitis with periodical presence of purulent discharge, photophobia and burning sensations. The diagnosis of pemphigus vulgaris was confirmed by histopathology, direct immunofluorescence and detection of anti-desmogelin 3 antibodies in patients' serum. Treatment was introduced with prednisone at a dose of 80 mg per day (1 mg/kg) and cyclophosphamide at a dose of 100 mg daily (1.25 mg/kg). After 7 days of therapy a significant reduction of eye symptoms was observed and after 4 weeks of treatment full clinical remission was achieved. Conclusions: 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.