Liver malignancy is known to be associated with hepatolithiasis. The present report summarises the results and our experience for management of 23 patients with intrahepatic hepatolithiasis associated cholangiocarcinoma (IHHCC). The correct diagnosis rates of US (ultrasonography), CT (computed tomography), and MRCP (magnetic resonance cholangiopancreatography) were 82.6% (19/23), 95.7% (22/23), and 91.7% (11/12), respectively. Carbohydrate antigen 19-9 (CA 19-9) was helpful in the diagnosis of IHHCC. All 23 patients with IHHCC underwent laparotomy. The surgical procedure consisted hepatectomy with a bile duct exploration in 16 patients (69.6%), a hepatectomy and drainage procedure such as sphincteroplasty and choledo-jejunostomy in three patients (13.0%), a bile duct exploration with biopsy in two patients (8.7%), and simple laparotomy and biopsy in two patients (8.7%). All the IHHCC patients who underwent a palliative procedure or laparotomy died within 1 year, and the overall cumulative survival rates at 1, 3, and 5 years were 43.8% (10/23), 13.0% (3/23), and 4.3% (1/23), respectively, and those patients who underwent curative resection were 88.9% (8/9), 33.3% (3/9), and 11.1% (1/9), respectively, which significantly longer than those (20.0%, 2/10; 0.0%, 0/10; and 0.0%, 0/10) patients who underwent palliative resection, respectively (p < 0.05). A suspicion of malignancy is necessary when managing patients with long-term hepatolithiasis. Hepatic resection with postoperative treatment is the treatment of choice for cholangiocarcinoma when it is resectable.