A 60-year-old woman presented with painful erosions in the oral mucosa, pharynx, perineum and perianal area, and multiple plaques with thick adherent crusts on the scalp. Most (nine) of the patient's fingernails had alterations in colour, affecting more than half of the nail plate, and all the toenails had severe inflammation of the nail folds, haemorrhagic paronychia and subungual or intraungual haemorrhage. A diagnosis of pemphigus vulgaris (PV) was made based on histology and on direct and indirect immunofluorescence findings. Groups of acantholytic cells were also observed in a Tzanck smear obtained from a subungual lesion. Onychomadesis in most of the fingernails and in all the toenails developed gradually. The patient was hospitalized and treated with oral corticosteroids. Complete recovery without residual damage to the nails and persistent remission was achieved. Nail involvement in PV is rarely described and is always of interest, as its presentation varies widely.