OBJECTIVE The etiology of jugular bulb stenosis (JBS) or occlusion in the context of vein of Galen aneurysmal malformations (VGAMs) is unknown. It can lead to decompensation of a lesion that was previously clinically stable. The aim of this study was to describe the natural history of JBS or occlusion in VGAM and to determine whether there is an association with bony remodeling of the jugular foramina. METHODS The authors identified all cases of JBS greater than 70% bilaterally involving patients seen at The Hospital for Sick Children between January 2007 and June 2014. The foramen diameters were measured on sagittal CT imaging, on a slice passing at the level of the jugular vein. The jugular foramen diameters were also compared to measurements obtained in a matched population of the same age group who had no VGAM and had undergone cerebral CT for a reason other than vascular disease. RESULTS Eight patients (6 male and 2 female) with bilateral JBS were included in this series. The median duration of clinical follow-up was 2.5 years (IQR 1.7-4.2 years). JBS was associated with bony narrowing the jugular foramina in 7 of the 8 patients over time. Between 1 and 2 years of age, patients with a VGAM demonstrated jugular foramen narrowing in comparison with a matched population (p = 0.015). CONCLUSIONS Jugular bulb stenosis or occlusion in VGAM may be associated with narrowing of the jugular foramina. These conditions seem to have a male predominance. If treatment is required, bony narrowing of the jugular foramina should be taken into account when deciding whether angioplasty and stent placement or surgical bypass might be appropriate therapeutic options.