<p class="abstract">Keratolytic Winter Erythema, also known as ‘Erythrokeratolysis hiemalis’ or ‘Oudtshoorn disease’ is a rare genetic disorder of keratinization of an unknown etiology characterized by cyclical erythema and intermittent skin peeling usually over palms and soles, particularly during winter.It was originally described in South African families of European descent originating from the Oudtshoorn district of Cape Provence, and has been since identified in several other countries. It is an autosomal dominant disorder with variable penetrance. The condition <a name="_Hlk67093214"></a>is often misdiagnosed as Keratolysis exfoliativa, but presence of a relevant family history, winter exacerbation and preceding hyperhidrosis with interdigital involvement differentiate it from the former. We present a case report with clinical histopathological data of an 18-year-old female with attributes of erthrokeratolysis hiemalis. This case is being reported due to its rarity.</p>