The Gorham-Stout disease is a very rare condition, characterized by lymphovascular proliferation and massive bone resorption. We present a 48-year-old male patient with osteolysis involving the left femoral head and neck, as well as to the ipsilateral acetabulum. Besides the morphological imaging, he underwent bone scintigraphy, technetium-99m-V-dimercaptosuccinic acid [99mTc(V)-DMSA] single photon emission computed tomography/computed tomography (SPECT/CT) and histological examination. Together these findings gave the definitive diagnosis. This is the first case ever published with 99mTc(V)-DMSA SPECT-CT. Advances on the knowledge of disease suggests that this imaging procedure could have utility in diagnosis and evaluation of the disease activity and therapy response.