A 34-year-old female patient was admitted to our hospital with a 1-year history of chronic congestive heart failure, massive proteinuria and tibial edema. On admission, she presented with hemolytic anemia, hepatomegaly, splenomegaly and renal impairment. Furthermore, the skin of her face, hand, forearm, lower extremities showed crust and bulla. Laboratory examination revealed a large amount of protoporphyrin in her blood and feces, but no increase in urine. Light microscopy of renal biopsy showed moderate chronic tubulointerstitinal disease and mild proliferation of mesangial cells. The prophyians are a group of compounds associated with involving the disturbance of various biosynthetic heme pathwas. Until now, the regulation of porphyrin and heme metabolism in the kidney have received relatively little attention as compared with those in liver and erythropoietic tissue. However, some recent reports have confirmed that proximal tubular cells may contribute to heme biosynthesis. It was strongly suggested that chronic tubulointerstitinal injury in this case might be directly induced by the disturbance of the biosynthetic heme pathway in the tubules.