Glomus jugulare tumor (GJT) is a rare benign neoplasm that is located in the skull base; it is impossible for ultrasonography to detect it in most cases. In this article, we present the case of a rare giant GJT, which extended into the internal jugular vein in the neck, initially detected by ultrasonography and confirmed by MRI and pathology. We highlight the initial ultrasonography findings and review the literature to summarize the ultrasonographic features of GJT.