Low-grade myofibroblastic sarcoma is a malignant tumor of myofibroblasts, which has only recently become more clearly defined. It represents a rare entity that progresses in a slow-growing infiltrative pattern inside deep soft tissues. Due to its rarity and the plasticity of the myofibroblast, it can cause significant diagnostic difficulties. Differencing this neoplasm from other spindle cell tumors requires the use of ancillary techniques such as immunohistochemistry and/or electron microscopy. The authors report an unusual case of low-grade myofibroblastic sarcoma of the larynx in a 24-year-old woman, with atypical clinicopathologic presentation. The patient underwent direct laryngoscopy with excision of the malignant mass followed by adjuvant radiation therapy. The authors emphasize the uncommon location of this tumor type and discuss management options.