We report the use of endoscopic endonasal surgery for dural reconstruction following a cerebrospinal fluid leak in a 33-year-old patient with recurrent meningitis since at age of 2 years. Magnetic resonance imaging showed osteolytic changes in the left temporal and sphenoid bones, including the left pterygoid plate, a few left temporal encephaloceles, and cerebrospinal fluid-like fluid in the expanded Meckel's cave and the parapharyngeal space. After endoscopic endonasal surgery, Gorham-Stout disease was diagnosed. No recurrence of cerebrospinal fluid leakage or meningitis has been observed. Thus, endoscopic endonasal surgery might improve clinical outcomes in patients with Gorham-Stout disease and skull base defects.