Punctate palmoplantar keratoderma (PPPK) is a rare entity with an estimated prevalence rate of about 1.17 per 100 000. The exact etiology of the disorder is not known but a dual influence of genetic and environmental factors may trigger the disease. We report the case of a 70-year-old mate patient with punctate palmoplantar keratodermic lesions for more than 40 years. Histopathologic examination revealed a hyperkeratotic epidermis without columns of parakeratosis or elastorhexis. On electron microscopy, the basal cells of the epidermis were found to have enlarged nucleoli and abundant tonofilaments, with keratohyalin-like granules confined to the upper part of the stratum spinosum, findings that were consistent with PPPK. Topical keratolytic agents were used with little success. Patients with PPPK and their next of kin should be investigated for possible associated malignancies.