Although liposarcomas are the most common type of sarcoma in adults, they are rare in the head and neck region. Pleomorphic liposarcoma is the least common histologic subtype in all locations. To our knowledge, there have been only 11 reported cases of primary liposarcomas of the major salivary glands, and the pleomorphic variant arising in the parotid gland has been reported only once before. Only 2 of the 11 reported cases of liposarcoma of the major salivary glands in the literature have died of disease, and both cases were of the pleomorphic subtype. We report a case of pleomorphic liposarcoma arising in the left parotid gland of an 80-year-old woman. This case report highlights that, despite conservative surgery followed by 2 local recurrences, our patient is doing well with minimal local morbidity and no demonstrable metastases 30 months after the initial diagnosis. We also present a review of the literature on the subject. (Am J Otolaryngol 2004;25:432-437.)