We present a case of perforating dermatosis in a patient with uraemic pruritus who was on haemodialysis and who was successfully treated with nemolizumab, a humanized monoclonal antibody targeted against interleukin-31 receptor A. Nemolizumab may be a treatment option for perforating dermatosis with a uraemic pruritus background. To our knowledge, this is the first report to show the effect of nemolizumab on perforating dermatosis.