BACKGROUND: Congenital disorders of glycosylation (CDG) are a group of metabolic disorders related to dysfunctional glycoprotein and glycolipid biosynthesis. ALG11-related CDG is a rare member of this group, characterized by severe neurodevelopmental impairment, progressive microcephaly, sensorineural hearing loss, and epilepsy. The objective of this report is to provide an update on the phenotype and brain magnetic resonance imaging (MRI) at age seven years for a patient initially described in early infancy with fetal brain disruption sequence. METHODS: We provide an updated detailed clinical description of a seven-year-old male with ALG-11 CDG who underwent brain MRI at age seven years. RESULTS: Brain MRI at age seven years showed significant disease progression compared to the neonatal brain MRI. There was near complete loss of cerebral hemispheres, severe cerebellar atrophy, and decreased volume of the brainstem. The prior brain MRI (done at six weeks of age) had shown severe supratentorial volume loss but a relatively preserved cerebellum and brainstem at that time. CONCLUSIONS: Reports on the natural history of rare conditions are important to improve our understanding of these conditions. ALG11-CDG is associated with atrophy and eventual vanishing of supratentorial brain structures, and infratentorial brain structures later in the disease process. The involvement of a pediatric palliative care service is a valuable adjunct to assist with symptom management and family support for these complex progressive conditions.