<p>Aquagenic papulotranslucent acrokeratoderma is a rare genodermatosis characterized by excessive wrinkling, a localized palmoplantar pruritic or a burning sensation, and asymptomatic yellowish-white translucent papules on the palms and soles following water exposure. It is often linked with cystic fibrosis. We delineate a possible new association, review this recognized cutaneous marker for cystic fibrosis plus describe a family not positive for cystic fibrosis or its carrier state but rather for another entity. We delineate a family with papulotranslucent acrokeratoderma that showed steroid-resistant nephrotic syndrome, a previously unidentified association, rather than cystic fibrosis or is carrier state. Only one family was identified and described. We suggest that aquagenic papulotranslucent acrokeratoderma may represent a potential cutaneous clue of steroid-resistant nephrotic syndrome, a rapidly progressive end stage renal disease.</p>