Acute paronychia, presenting as abscess formation involving the paronychium of the nails, is thought to be an uncommon feature of pemphigus vulgaris (PV). We report a patient with PV who developed acute paronychia of multiple fingernails and toenails, that precede to the exacerbation of other mucocutaneous lesions. A biopsy specimen of the paronychium of the finger revealed suprabasal acantholysis with pustule formation. Direct immunofluorescence study of the same lesion showed intercellular deposition of immunoglobulin (Ig) G and C3. Systemic corticosteroids and azathioprine were effective in treating both the nail disease and mucocutaneous lesions. We concluded that in patients with PV, acute paronychia could be a manifestation of the disease per se, rather than an infectious process.