A 74-year-old woman was transferred to our hospital for further examinations because of abdominal fullness and abnormal levels of serum liver/biliary enzyme persisting for 3 weeks. She had anemia and dilatation of many capillary vessels in her fingers, palms, and tongue in addition to reporting frequent incidences of nasal bleeding in herself and her family. Abdominal ultrasonography detected a cystic lesion in the right hepatic lobe, connected to a dilated tortuous hepatic artery. A low-echoic hepatic phyma was also detected in the back of the cystic lesion. Abdominal computed tomography and magnetic resonance imaging indicated that the cystic lesion was an aneurysm and the low-echoic phyma was a hematoma. Hepatic arteriography confirmed a hepatic aneurysm, tortuous dilatation of the hepatic artery, and the complication of an arteriovenous shunt in the liver. Taking all of these findings into consideration, this case was diagnosed as hereditary hemorrhagic telangiectasia (HTT) complicated by a hepatic aneurysm causing intrahepatic hematoma. To prevent re-rupture of the aneurysm, we performed a hepatic arterial coil embolization. After therapy, no blood flow to the aneurysm was detected by ultrasonic color Doppler method and the hematoma gradually diminished. There have been no reports of a case in which hepatic arterial embolization was effective for HHT-associated hepatic aneurysm causing intrahepatic hematoma. This very rare case provides important clinical information regarding abdominal vascular complications of HTT and a less invasive treatment for them.