Acute intermittent porphyria (AIP)is a genetic disorder of haem biosynthetic pathway characterised by increased levels of porphyrin precursors –delta aminolevulonic acid (ALA)and porphobilinogen (PBG).Previous studies have suggested an association between hepatocellular carcinoma (HCC)and acute hepatic porphyries .The important feature of AIP is the risk of development of acute crises which can be precipitated by a variety of factors like certein drugs,infections, or surgical operations. We discribed a case of hepatectomy due to hepatocellular carcinoma in 70-year-old woman with acute intermittent porphyria. The diagnosis was performed during rutine ultrasound of the abdomen.It was described in CT as well separated 75x62 mm size tumor in segment 4 of the liver. A high level of protoporphyrins was noticed before surgery. Resection of the 4th segment of the liver and cholecystectomy were performed. There was no complications during surgery nor in the postoperative period. Conclusions: 1.Periodic screaning for hepatocellular carcinoma should be evaluated in patients with acute intermittent porphyria. 2.Safe surgery can be performed in patients with acute intermittent porphyria despite of high level of porphyrin precursors.