Objective To investigate the diagnosis and operation treatment of primary intrahepatic cholangiocelhlar carcinoma (PICC), for improving the level of diagnosis and treament of PICC. Methods The clinical data of 18 cases with PICC confirmed by operation were analyzed retrospectively. Results In the early stage, no specific symptoms was found in all the 18 cases, the positive cases of AFP, CEA, CA199 and live cirrhosis were 2, 4, 3 and 4. The diagnostic rates of ultrasound examination, CT and MRI were 11.1%(2/18), 42.9%(6/14) and 45.5% (5/11 ). Seven cases were diagnosed as suffering from PICC and the others were misdiagnosed. Of all the 18 patients, 8 cases underwent radical resection and 10 cases received palliative excision. Conclusions PICC patients lack clinical features and serum tumor marker,the rato of misdiagnosis is high, but that of radical resection is low. Knowing its clinicopathological features well. Radicalresection is the best way for treatment of PICC.