Cholangiocarcinoma (CCA) is the second primary hepatic malignancy after hepatocellular carcinoma. CCA is characterized by its aggressiveness and poor prognosis associated with limited therapeutic options. Thus, a better understanding of molecular pathogenesis of CCA together with the identification of robust biomarkers and therapeutic targets is still necessary to improve the management of patients with CCA. In this review, we discuss the role of long non coding RNAs (lncRNAs) in CCA onset and progression. LncRNAs represent a heterogeneous class of RNA molecules with various genomic location and organization acting at both transcriptional and post-transcriptional levels. Notably, we provide an overview of the functions of lncRNAs in epigenetic modulation and regulation of signaling pathways and cell behaviors leading to tumor progression (e.g. apoptosis, survival, epithelial to mesenchymal transition, migration, invasion, angiogenesis). Moreover, we depict the clinical relevance of lncRNAs as biomarkers and emerging therapeutic targets in CCA.