Gorham-Stout disease (GSD) is a rare disorder marked by massive bone destruction. A 56-year-old woman with GSD presented to our hospital with a proximal femur fracture. She was initially diagnosed with a simple fracture and underwent open reduction and internal fixation using an intramedullary nail. However, the delayed diagnosis of GSD resulted in postponed appropriate treatment. Although the intramedullary nail helped prevent mechanical bone failure, extensive osteolysis continued, ultimately necessitating total femur replacement. This case highlights the importance of considering GSD as a potential diagnosis of extensive osteolysis to avoid delays in treatment and subsequent severe bone loss.