Patients with Gorham–Stout disease (GSD) present progressive destruction and resorption of bone.
Typical bone-related symptoms include swelling, pain and functional impairment in the region involved.
The three aspects of GSD etiopathology are osteoclasts, angiogenesis/lymphangiogenesis and osteoblast function.
Multi-targeted pharmacological approach includes innovative options and represent milestones of treatment, sometimes associated with radiotherapy.
Surgery is mainly used to treat complications: pathologic/impending fractures, spinal instability or deformities and chylothorax.
In this narrative review, we highlight current standards in diagnosis, clinical management and therapeutic strategies.