A case of severe familiar erythropoetic protoporphyria is described. It seems to be obvious, that in this case, where an incomplete obstruction of the biliary tract led to a high porphyrinuria (3,5 mg/day) in connection with a highly increased liver-δ-aminolevulic-acid-synthetase-activity and extremely high content of liver-porphyrins (77 mg%), the liver takes an important part in this disfunction of porphyrin metabolism, formerly believed to be a purely erythroblastic disease.