Pleomorphic adenoma is the most common benign tumor of salivary glands. Eighty percent of pleomorphic adenomas occur in the parotid gland, 10% in the submandibular gland, and 10% in minor salivary glands and sublingual glands. In this case report, we aim to present the unusually giant presentation of submandibular gland pleomorphic adenoma and its management of a 52-year-old female patient who presented to us with a swelling in the right submandibular region for the last 5 years. Fine needle aspiration cytology (FNAC) suggested salivary gland origin. Ultrasonography of the neck revealed a well-defined large lobulated heterogeneous echotexture lesion with few cystic areas within and showing internal vascularity in the right submandibular region. Computed tomography (CT) scan revealed a large lobulated heterogeneously enhancing mass of 15 cm × 13 cm in the right submandibular region. The patient underwent right submandibular gland mass excision under general anesthesia. The patient presented good aesthetic and functional results with no clinical and radiographic evidence of recurrence. Being painless and slowly growing, some patients neglect it to such large sizes especially if surgery was a psychological setback for them which can then lead to facial disfigurement and severe pressure symptoms which may be fatal. Giant pleomorphic adenoma of the submandibular gland is a rare finding and should be diagnosed cautiously. Proper treatment is complete surgical excision with removal of the fibrous capsule so as to prevent recurrence.