Hepatocellular carcinoma (HCC) with acquired porphyria is a very rare condition. It is characterized variably by hyperpigmentation, skin fragility and photodistributed subepidermal vesicles. The serum, urine and/or stool porphyrin levels, usually markedly elevated, can change according to the clinical course. We report here a case of hepatocellular carcinoma presenting with a paraneoplastic syndrome of acquired porphyria. A 73-year-old Chinese woman had the characteristic facial pigmentation of cutaneous porphyria and histologically proven hepatocellular carcinoma. Her serum zinc protoporphyrin was elevated and her urine tested positive for coproporphyrin. Her protoporphyrin and alpha-fetoprotein levels dropped after transarterial chemoembolization treatment. Acquired porphyria in hepatocellular carcinoma occurs exclusively in older persons with huge hepatocellular carcinoma and/or cirrhosis. Before diagnosis, it must be carefully differentiated from inherent porphyrias with HCC, and porphyrias induced by drugs or heavy metal intoxication must be ruled out.