Malignant salivary gland sarcomas represent a clinically and histologically diagnostic challenge. Primary unclassified sarcomas of the parotid gland consist a rare salivary gland tumor. We report an unusual case of such a tumor, which occurred in the right parotid gland of a 54-year-old male and presented as an asymptomatic painless mass. The pathologoanatomical examination revealed a rhabdoid large-cell unclassified sarcoma. The patient was treated with superficial parotidectomy and adjuvant radiotherapy. No recurrence was noted in a 10-year follow-up period. Due to the rare occurrence of primary unclassified sarcomas, there is no evidence-based treatment of choice. An optimal approach is best planned in a multidisciplinary setting, taking into consideration the resectability of the tumor, individual patient characteristics, presence of local or distant metastatic activity, local infiltrative behavior and tumor stage. A close follow-up of the patient is strongly recommended.
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