Carcinoma ex pleomorphic adenoma (Ca-ex-PA) is a rare tumor that arises from the malignant transformation of a primary or recurrent pleomorphic adenoma. Despite being benign, pleomorphic adenomas can rarely undergo malignant transformation. Risk factors include a long-standing primary tumor, a prior history of radiation exposure, increased tumor size, and recurrent disease. Ca-ex-PA usually affects patients between the sixth and eighth decades of life, approximately 10 to 20 years after the development of a pleomorphic adenoma. Patients usually present with the rapid expansion of an already existing mass. We describe a case report of a patient who presented with Ca-ex-PA of the submandibular gland. The patient underwent surgical excision of the affected gland, which was consistent with a widely invasive myoepithelial Ca-ex-PA. The patient underwent postoperative radiation to the neck and the tumor bed. No local or distant recurrence was noted during the one-year follow-up. Due to the rarity of the disease entity and the infrequent location of the tumor, this case presents a particular diagnostic and therapeutic challenge.