Gorham-Stout (GS) disease is a rare disease of the bone and is also known as massive osteolysis. Less than 200 cases have been reported in the world literature. A 29-year-old female with a diagnosis of GS disease was identified. She complained of aural fullness and tinnitus bilaterally. Demineralization and moth-eaten changes of the osseous structures of the skull base and posterior fossa were prominent. The left mastoid air cells were opacified and erosion extended to the left jugular foramen, left hypoglossal canal, left stylomastoid process, and left eustachian tube. The radiographic findings and brief literature review are presented.