Patients with nephrogenic systemic fibrosis may manifest a range of cutaneous lesions. The skin may be variably affected with subtle, superficial papules and plaques; deeper dermal or subcutaneous induration; or severe contractures of the joints. Recognition of the cutaneous manifestations of nephrogenic systemic fibrosis, and consideration of the differential diagnosis of such lesions, facilitates diagnostic confirmation and lends insight into the pathogenesis of this disorder by comparison with clinically similar conditions. This review considers the dermatologist's perspective on nephrogenic systemic fibrosis, including the variety of skin findings and the potential for treatment with extracorporeal photopheresis.