Objective: To investigate the clinical features of intrahepatic cholangiocarcinoma (ICC), for increasing the understanding of this disease, and reducing its misdiagnosis and inappropriate treatment. Methods: The clinical date of 42 ICC patients (ICC group) and 80 hepatocellular carcinoma (HCC) patients (HCC group) with pathological confirmation, admitted from January 2005 to December 2013, were retrospectively analyzed. Results: In ICC group compared with HCC group, there was no gender difference (number of male cases was more than female cases in HCC group); the number of cases with past or present cholelithiasis was increased, and most stones were on the same side of the tumors; the positive rate of HbsAg and AFP as well as their detection values were decreased, while the positive rate of CA19-9 and proportion of cases with high γ-GT level as well as their detection values were increased; the imaging profiles were more characterized by enlargement of abdominal lymph nodes, bile duct stones, peritumoral bile duct dilation and liver capsule retraction or lobe atrophy; the preoperative misdiagnosis rate was high, tumor cells showed histologically poor differentiation, and abdominal lymph node metastasis rate was increased; liver tumor resection plus abdominal lymphadenectomy was the most adopted procedure. All the difference had statistical significance (all P<0.05). Conclusion: ICC has some clinical features different from those of HCC, and a better understanding of ICC may reduce its misdiagnosis and wrong treatment.