Background Sarcomatoid carcinoma (SC) is a rare variant of squamous cell carcinoma (SCC). It is a malignant tumor containing an SCC (in situ or invasive) component and a predominantly sarcomatous component with malignant spindle and/or pleomorphic cells. It accounts for less than 1% of all laryngeal tumors. Case Report An 8 × 5 mm polypoid lesion on the right vocal cord of a 72-year-old man with a history of smoking who presented with hoarseness was excised. In microscopic examination of the excision material, a tumoral lesion consisting highly pleomorphic cells with large nuclei and prominent nucleoli that had a spindle cell morphology in some places, an epithelioid cell morphology in some areas, and occasionally a giant cell morphology was observed. The tumor surface had an ulcerous appearance in most areas. There were typical and atypical mitoses in the tumor. Although squamous epithelium in intact benign appearance was in an area on the tumor surface, the presence of squamous epithelium containing atypical cells with disrupted organization standing apart from the tumor was remarkable. Immunohistochemical analysis revealed positive staining with smooth muscle actin and vimentin in the tumor cells. Multifocal positive staining with Pan-CK, EMA, and P40 was observed in the squamous epithelium and lesion. CD34 and desmin were negative. The case was reported to be compatible with SC. Conclusions SC is a rare variant of SCC. In the differential diagnosis, many tumors in spindle cell-like appearance such as nodular fasciitis, spindle cell melanoma, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumors should be kept in mind and examined carefully. Sarcomatoid carcinoma (SC) is a rare variant of squamous cell carcinoma (SCC). It is a malignant tumor containing an SCC (in situ or invasive) component and a predominantly sarcomatous component with malignant spindle and/or pleomorphic cells. It accounts for less than 1% of all laryngeal tumors. An 8 × 5 mm polypoid lesion on the right vocal cord of a 72-year-old man with a history of smoking who presented with hoarseness was excised. In microscopic examination of the excision material, a tumoral lesion consisting highly pleomorphic cells with large nuclei and prominent nucleoli that had a spindle cell morphology in some places, an epithelioid cell morphology in some areas, and occasionally a giant cell morphology was observed. The tumor surface had an ulcerous appearance in most areas. There were typical and atypical mitoses in the tumor. Although squamous epithelium in intact benign appearance was in an area on the tumor surface, the presence of squamous epithelium containing atypical cells with disrupted organization standing apart from the tumor was remarkable. Immunohistochemical analysis revealed positive staining with smooth muscle actin and vimentin in the tumor cells. Multifocal positive staining with Pan-CK, EMA, and P40 was observed in the squamous epithelium and lesion. CD34 and desmin were negative. The case was reported to be compatible with SC. SC is a rare variant of SCC. In the differential diagnosis, many tumors in spindle cell-like appearance such as nodular fasciitis, spindle cell melanoma, leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumors should be kept in mind and examined carefully.