A 26-year-old Korean man exhibited generalized lentigines, a slight prominence of the chin, short stature, and a familial history consistent with the autosomal dominant mode of inheritance. The multiple lentigines appeared at the age of 1 and spread over his entire body including the scalp, palms, and soles by the age of 4. There were several large dark pigmented spots on the trunk, but no other skin abnormalities were found. We performed histologic studies of the normal skin and the lentigo by both electron and light microscopy with immunohistochemical stains. Routine hematoxylin-eosin staining of the pigmented skin revealed heavily pigmented basal and spinous layers and prominent rete ridges. Fontana-Masson stain showed enormous black pigmentations on the basal and spinous layers of the pigmented macule. S-100 protein stain of the pigmented lesion demonstrated an increased number of melanocytes per unit skin area. Ultrastructural studies showed the presence of extensive aggregated melanosomes within the keratinocytes in the epidermis of lentigo, but not the presence of giant melanosomes in either normal skin or lentigines.