Mesenteric vasculopathies in pediatric patients pose a challenge for clinicians due to their nonspecific clinical presentations and the lack of reported clinical experience. Few cases have been reported in literature and knowledge of mesenteric vasculopathies has not been thoroughly understood. Diagnosis and management of mesenteric vasculopathies in children is heavily dependent on proper radiologic imaging. In this review, we attempt to consolidate information regarding the clinical presentation, diagnosis, and management of mesenteric vasculopathies of congenital malformations, genetic anomalies, vasculitis, and ischemic insults. Pediatric interventional radiology is providing a forefront of minimally invasive techniques for both diagnosis and treatment of various pathologies of the mesenteric vasculature.