Aneurysmal bone cyst (ABC) is an expansile bone lesion that usually involves the long bones. Skull base involvement is rare. Hereby, we describe a 17-year-old man with hoarseness, facial asymmetry, left sided sensorineural hearing loss and left jugular foramen syndrome. CT scan and MRI showed a skull base mass that was confirmed as ABC in histopathology. The case was unusual and interesting due to the clinical presentation of jugular foramen syndrome and radiological findings such as severe enhancement and multiosseous involvement.