Background: Epidermal malformations of the skin include a group of heterogeneous developmental defects that result from errors in morphogenesis of the epidermis during intrauterine life. Objective: The purpose of this study was to report the clinical and histopathologic features of a distinctive epidermal malformation involving the skin of the palms or soles. Methods: Ten patients were included in this study. All of them showed the same clinical features that consisted of a solitary circumscribed and circular area of erythematous depressed skin on the palm or on the sole. Diagnosis was confirmed by histopathologic study. Results: All patients were middle aged or elderly. Nine patients were women and one was a man. The lesions showed predilection for the skin of the thenar and hypothenar regions of the palm or the medial side of the sole. Histopathologic study demonstrated a depression of the epidermis, with a sharp stair between normal and involved skin. The epidermis covering the depression showed markedly thinner horny layer and a slightly diminished granular cell layer when compared with adjacent noninvolved skin. Keratinocytes of the squamous cell layer, granular cells, and corneocytes showed, otherwise, a normal appearance. Serial sections failed to demonstrate cornoid lamellation. Conclusion: On the basis of the clinical and histopathologic findings in these 10 patients, we have named this malformation circumscribed palmar or plantar hypokeratosis. This lesion seems to be a distinctive entity that has not been previously described.