Plexiform fibromyxoma (PFM) is a rare gastrointestinal tumor, primarily found in the stomach. Esophageal PFM is exceptionally rare. We describe a case of a mid-20s woman with respiratory and swallowing difficulties, revealing a 105x65 mm upper thoracic esophageal submucosal tumor during endoscopy. Biopsy lacked histological evidence of gastrointestinal stromal tumors (GISTs). Post-tumor removal histopathology showed a spindle tumor with plexiform architecture and myxoid-vascular stroma. Immunohistochemistry revealed vimentin and alpha-smooth muscle actin expression, while desmin, c-kit, DOG1, and CD34 were absent, confirming PFM. No recurrence or metastasis appeared during a 6-month follow-up. This case underscores the extreme rarity of esophageal PFM, emphasizing the need for precise diagnostic tools to navigate challenging differential diagnosis.