Objective: To demonstrate a general view on the clinical and morphological aspects in children with Hydranencephaly. Methodology: This is a study that uses the systematic review as a method. Data collection took place in September 2019 through the SciELO and EBESCO databases with the following descriptors:"hemihydranencephaly", '' Rare diseases '', 'malformation'. For the selection of articles, the following were used: inclusion criteria: articles in Portuguese, English, full articles that portray the theme related to the literature review and articles published and indexed in the referred databases in the period from 2007 to 2019. Results and Discussion: Hydranencephaly is a condition that in Most are lethal with exceptions, this malformation of the central nervous system occurs after the closure of the neural tube, defined as total or partial absence of cerebral hemispheres, the entire cortex that would be irrigated by the carotids is nonexistent. however, the brainstem, diencephalon and part of the occipital cortex, irrigated by the posterior cerebral arteries, are formed and may exhibit electrical activity. In some cases they have severe neurological sequelae, psychomotor disability, visual impairment, deafness and progressive macroencephaly. Conclusion: Patients with this rare condition are subjected to several surgeries and antibiotic therapies for prolonged periods in an attempt to provide the best survival for the patient. irrigated by the posterior cerebral arteries, are formed and may exhibit electrical activity. In some cases they have severe neurological sequelae, psychomotor disability, visual impairment, deafness and progressive macroencephaly. Conclusion: Patients with this rare condition are subjected to several surgeries and antibiotic therapies for prolonged periods in an attempt to provide the best survival for the patient. irrigated by the posterior cerebral arteries, are formed and may exhibit electrical activity. In some cases they have severe neurological sequelae, psychomotor disability, visual impairment, deafness and progressive macroencephaly. Conclusion: Patients with this rare condition are subjected to several surgeries and antibiotic therapies for prolonged periods in an attempt to provide the best survival for the patient.