Introduction. Squamous cell carcinoma and sarcoma are malignant tumours, which can appear in different parts of the body. Spindle cell carcinoma (SCC), in terms of histopathology, is a type of cancer that has both spindle cell carcinoma and sarcoma features. Case Report. The case is presented of a 45-year-old-man with diagnosed spindle cell carcinoma which occurred on the right side of the patient's maxilla. This type of tumour is really rare for the area of the head and neck. The patient has been diagnosed using dental volumetric tomography, Cone Beam Computed Tomography (CBCT) and bone biopsy. The patient has undergone comprehensive treatment, which includes radical resection of the tumour area as well as chemotherapy and radiotherapy. Regular control visits and diagnosis after completed treatment are extremely important for the final outcome of treatment.