Cyclic vomiting syndrome (CVS) is a syndrome characterized by stereotypical, recurrent episodes of severe nausea and vomiting. Episodes can last from a few hours to several days, and it returns to baseline health during inter-episodic phase. Since the pathogenesis is unknown, and there are no specific biomarkers available for this disorder, the diagnosis often relies on clinical features. Due to the aforementioned reasons, delayed diagnosis is often caused. The Rome IV is the most used criteria for CVS diagnosis. But so far, no standard therapeutic regimen currently exists to manage CVS, and the treatment is primarily empirical. This review mainly summarizes the diagnosis and treatment of CVS.