Hereditary punctate palmoplantar keratoderma (Buschke-Fischer-Brauer disease) is a rare disorder of keratinization. We describe here a 49-year-old male patient of this condition with many unusual features such as late onset of the disease in the fourth decade and nail changes (longitudinal pigmented striations, curved nails and pits in the fingernails; and nail thickening, subungual hyperkeratosis and yellowish discoloration in toenails). The patient developed histopathologically proven skin lesions typical of psoriasis 7 years after appearance of the keratoderma. This association has not been reported earlier. The patient's skin lesions cleared completely with acitretin therapy within 3 months.