The skin changes reported in patients with end-stage renal disease (ESRD) are diverse and manifold. In this article we focus on a collection of specific cutaneous entities seen most frequently in the setting of ESRD, each presenting with distinctive and unique morphology. These include perforating disorders, porphyria cutanea tarda, pseudoporphyria, calcinosis cutis, calciphylaxis, and nephrogenic systemic fibrosis. The clinical features, histopathology, pathophysiology, differential diagnosis, and management of each entity are reviewed.