Objectives: This review aims to evaluate the efficacy of the combination of dexmedetomidine and ketamine (KD) and focuses on pediatric perioperative and periprocedural applications of KD as well as its limitations e adverseevents. Despite the concomitant use of these two drugs is well described in adult population, there are few studies on its administration in pediatric anesthesia. Discussion: Drug combination proves to be an attractive regimen when adverse effects of one agent counteract the effects of another. When used together, dexmedetomidine can prevent ketamine’s tachycardia, hypertension, sialorrhea and emergency agitation, while accelerating the onset of sedation, thus excluding prolonged dexmedetomidine latency as a single agent. Such profile may be favorable for pediatric patients in order to conceive adequate anxiolysis, prevention of emergency delirium, also allowing its use for procedures with significant algic stimulus. A literature search was conducted in PubMed, Lilacs and Embase to identify 21 articles from 2015 to 2019 that address the use of ketamine and dexmedetomidine simultaneously during anesthetic procedures in population between birth and18 years old. Conclusion: The literature is favorable to the use of KD for invasive and noninvasive procedures, inside and outside of the operating room, presenting an attractive profile for pediatric patients.