Temporal bone carcinomas are rare tumours with a variable presentation. Despite their rarity, they most commonly derive from the periauricular skin and the external auditory canal skin. The most common histological type of these tumours are squamous cell carcinoma, which tend to spread locally and lymphogenically to the parotid gland and the neck. The second most common histological type of tumours affecting this area are basal cell carcinoma, while other types are negligible in frequency. Diagnosis of malignant temporal bone tumours is based on detailed dermatological and neurootological examination and radiological imaging techniques. There is no generally accepted classification system for these tumours, but the modified Pittsburgh classification system from 2000 is most commonly used. The treatment of malignant tumours of the temporal bone is primarily surgical, and it is based on resections of the soft tissue of the external auditory canal and the temporal bone of various extents, as well as frequent superficial parotidectomy and neck dissection, depending on the indication. After the operation, depending on the extent of it, the reconstruction of the sacrificed facial nerve and facial musculature is performed. Surgery is often accompanied by adjuvant radiotherapy, which is proven to be beneficial for the patients' survival. In a recent research, concomitant chemoradiotherapy has also emerged as an option in advanced stages of the disease. The prognosis of these tumours largely depends on the stage of the disease, and for advanced stages it is extremely poor.