Necrolytic acral erythema (NAE) has been described as an early cutaneous marker for hepatitis C virus (HCV) infection. It most commonly presents as a well-defined, dusky, erythematous eruption with marked hyperkeratosis and a dark red rim associated with pruritus or burning. Necrolytic acral erythema bears microscopic and clinical resemblance to other necrolytic erythemas, including necrolytic migratory erythema (NME) and several nutrient-deficient syndromes. It is distinct, however, in its predominantly acral distribution and strong association with HCV infection. The pathogenesis is unknown, but a relationship to metabolic alterations has been hypothesized. Optimal therapy appears to be treatment of the underlying HCV infection using a combination of ribavirin and interferon alfa; oral zinc therapy may be an alternative but useful therapy. Cases of NAE without HCV infection suggest that more work needs to be done defining NAE and its relationship to HCV.