The purpose was to systematically review all published cases of Gorham-Stout disease (GSD) involving the jaws, identify the clinic-radiological and histopathological features associated with the persistence of the lesions, and the best treatment options available. An electronic search was undertaken in November/2018. Eligibility criteria included publications having enough information to confirm the diagnosis. Eighty-six publications reporting 89 cases were included. Features observed: symptomatic (51.1%), swelling (34.1%), pathological fracture (31.8%), history of previous trauma (32.1%), high levels of alkaline phosphatase (24.3%), predominance of vascular tissue (72.4%). Nearly 1/4 of the patients were only followed up and no treatment was implemented. Most of treatments consisted of some type of surgery with/without additional therapies (42.0%), drugs (20.5%), and radiotherapy (14.8%). Half of the cases were found to persist after some treatment modality and five patients died. Among the different variables investigated, only the fact of crossing the midline showed association with the persistence of the lesion. There is still a lot to understand about GSD, a rare condition with no clear consensus on the etiopathology, unpredictable clinical course, and no standard treatment. The high percentage of cases that persist after treatment are found to be associated only with the lesion crossing the midline.