Hereditary palmoplantar keratoderma, a well-known clinical entity, is illustrated through a familial report of an unmarried young man who is the product of a consanguineous marriage (paternal and maternal grandmothers were sisters). The lesions were characterized by immense yellow waxy thickening of the skin surrounded by erythematous border (halo) and fissures/cracks associated with extensive scaling of the palms and soles. The lesions were bilateral and symmetrical. These features were supported by orthokeratotic hyperkeratosis hypergranulosis and acanthosis in hematoxylin-eosin stained tissue sections prepared from the soles. Mycelia/spores could not be identified on Periodic acid-Schiff (PAS) reaction. An autosomal dominant trait was revealed through family pedigree. An abridged update to recap the current status is highlighted.