Recently, miRNA profiling studies have indicated that miRNA-196 (miR-196) is overexpressed in several tumour tissue samples. In addition, increasing numbers of reports indicate that miR-196 plays important roles in development and immunity through targeting of specific genes. In this review, we discuss the known functions and molecular mechanisms of miR-196 in normal development and the pathogenesis of cancer. Further investigation of these newly discovered functional roles of miR-196 may lead to potential clinical applications of miR-196 in the management of several human diseases.