The children who remain in a prolonged disorder of consciousness (PDOC) present a complex clinical, ethical, and legal challenge to health professionals and other caregivers. PDOC is defined as any disorder of consciousness that has continued for at least 4 weeks following sudden-onset brain injury. The PDOC includes the vegetative state/unresponsive wakefulness syndrome (EV/ UWS), and the minimally conscious state (MCS). Patients with PDOC lack of mental capacity to make decisions regarding their care and treatment, so these decisions have to be made for them based on their best benefits. These benefits may vary from patient to patient, between physicians, family, and the general public, creating conflict within their respective efforts to do what they belie ve is right for the patient. The diagnosis is based on clinical evaluations. These evaluations have an estimated misdiagnosis rate up to 45%, therefore they should be complemented with standardized clinical guidelines, and often with neuroimaging and neurophysiological studies. Other aspects that difficult the evaluation are variable definitions and subcategorizations of PDOC, among di fferent groups at the international level. The objective of this review is to present an update of the different types of PDOC, their definition, subcategorization, etiology, prognostic, comprehensive evaluation, and treatment in pediatrics, to contribute to the best clinical practice based on cu rrently available evidence.