We report here the imaging findings of a rare case of a lethal form of Gorham disease in a young female patient. Multimodality imaging findings over 13 year-follow-up demonstrated progressive wide spread skeletal and soft tissue abnormalities with permeative osteolysis, pathological fractures and severe skeletal deformities. Unusual extensive osseous and soft tissue pneumatosis was illustrated on cross-sectional studies. The progressive nature of this form of Gorham disease and the subsequent complications eventually culminated in patient's death.