<p>Gorham-Stout disease (GSD) is a rare disorder characterised by progressive osteolysis and angiomatous proliferation. Bone loss can be so rapid and extensive that it leads to the complete disappearance of entire skeletal segments, earning the disease the colloquial name of “phantom bone disease”. Despite advances in research, the pathogenesis of GSD remains partially unknown, and the molecular and cellular mechanisms underlying progressive osteolysis are not yet fully understood. This review aims to provide an overview of studies investigating alterations in bone remodelling in patients with GSD, with the goal of highlighting novel perspectives for the development of innovative diagnostic and therapeutic strategies. Furthermore, we discuss findings from molecular genetic studies and the currently available treatment options, emphasising future prospects for the management and therapy of the disease.</p>