An autopsy case of Dubin-Johnson syndrome (DJS), associated with hepatocellular carcinoma (HCC), the third such case in Japanese literature, is reported. A 64-year-old man, known to have had jaundice from his childhood, was admitted because of weight loss. Close examination revealed that the patient had DJS with HCC and a right lobectomy of the liver was performed. He died, however, of metastasis of HCC about 36 months later. At autopsy, the liver was found to be brownish-black in color. Microscopic findings of the liver were those common to HCC and chronic active hepatitis, and the brown pigment seen in the hepatocytes was mainly confined to the centrilobular ares. After histochemical and electronmicroscopic study, a close relation between this pigment and lysosome is suspected.