Objective: Juvenile myoclonic epilepsy (JME) is one of the most common childhood types of epilepsy and comprises 5-10% of all epilepsies. Altered expression levels of microRNAs (miRNAs) have been reported in epilepsy as in many diseases. As is known, miRNAs regulate gene expression post-transcriptionally and have potential as diagnostic biomarkers due to their stability in clinical samples. Herein, this study aimed to evaluate miR-1179 levels of JME patients and assess the potential of miR-1179 as a diagnostic biomarker. Materials and Methods: Twenty patients and 20 healthy controls were recruited in this study and total RNA was extracted from peripheral blood samples of participants. Quantitative real-time polymerase chain reaction (qRT-PCR) was performed to calculate the relative expression level of miR-1179. Additionally, receiver operating characteristic (ROC) curve was conducted to evaluate the diagnostic value of miR-1179 in JME. Results: Expression levels of miR-1179 were statistically significantly increased in patients with JME compared to healthy controls (p<0.0001). ROC analysis revealed that miR-1179 is a well diagnostic biomarker with an area under the curve (AUC) of 0.89. Conclusion: miR-1179 may be considered a remarkable biomarker in the diagnosis of JME. The interaction between miR-1179 and its target Calmodulin 1 (CALM1) should be reinforced through functional studies. Further research in larger cohorts will help to enlighten the etiopathogenesis of JME.