Nasal spindle cell rhabdomyosarcoma is very rare. The tumor is sometimes confused with other spindle cell tumors. We herein report a case of nasal spindle cell tumor in a 62-year-old woman. The patient first presented herself to a medical doctor's office after an episode of left epistaxis. An intranasal tumor was found and resected. The tumor was composed of spindle cells, and she was diagnosed with desmoid-type fibromatosis. Five years after the initial episode, an intranasal tumor was found again. The tumor showed a fascicular growth pattern with high cellularity and was predominantly composed of spindle cells. Scattered eosinophilic rhabdomyoblasts were also observed. She was diagnosed with spindle cell rhabdomyosarcoma. This is a unique case report not only because nasal spindle cell rhabdomyosarcoma is very rare but also because the tumor was initially diagnosed as desmoid-type fibromatosis. It is important to consider spindle cell rhabdomyosarcoma as a differential diagnosis of nasal spindle cell tumors.