An intrathoracic meningocele was diagnosed in a 37-year-old woman who had the clinical features of neurofibromatosis type 1 (NF-1), including café-au-lait spots, cutaneous neurofibromas and kyphoscoliosis. Chest radiography of this patient revealed a huge mass-like radiopaque density or consolidation in the left upper lung field. In these cases, meningocele should be differentiated from posterior mediastinal tumors such as neurofibroma, neuroblastoma, and ganglioneuroma, because NF-1 has a relatively high risk of tumor formation. Regular follow-up with periodic imaging and without surgical treatment was recommended.