Clinical phenotype during prodromal, vomiting, and recovery phases contains visceral and neuropsychological symptoms, but also cranial and systemic symptoms. Some clinical arguments as circadian or circannual periodicity suggest a chronobiological disease. Red flags in clinical presentation are proposed to distinguish other etiologies of recurrent gastrointestinal disturbances and guide paraclinical explorations. Functional magnetic resonance imaging in both CVS and migraine displayed diminished insular connectivity with the sensorimotor network, suggesting a common pathophysiology. Pathophysiology of CVS is not well defined, and there is probably a multifactorial origin. Distinction with other differential diagnoses is a challenge for clinicians. Further research, in particular with functional imaging, are required to define pathophysiology of CVS. Control trials are missing in pediatric population. Injectable or intranasal sumatriptan are often effective. For prophylaxis, amitriptyline, cyproheptadine, or propranolol are the most common treatments, depending on age and comorbidities. Non-pharmacologic measures as lifestyle modification also seem to be effective as preventive treatment.