Aim: To discuss the clinical presentation of a primary hydatid cyst of the submandibular gland and its surgical implications. Background: Hydatid cyst disease is a parasitic, cyclo-zoonotic infestation by cestode of the genus Echinococcus. In humans, the most commonly affected organs are the liver (70%) and lungs (20%) followed by spleen, muscles, bones, kidneys, and the central nervous system. Primary hydatid cyst of the head and neck region; however, is extremely rare and very few cases have been reported in literature so far. Case description: We report the case of a 28-year-old male who presented with a 3-year history of a gradually increasing swelling over the right submandibular region. It was firm, painless with no other relevant features. There was no history of exposure to farm animals or ingestion of tainted meat. Ultrasonography of the neck revealed a simple anechoic cyst followed by fine needle aspiration cytology (FNAC) which confirmed the diagnosis of a hydatid cyst. The patient underwent submandibular gland excision under general anesthesia wherein the entire gland with the cyst was excised in toto. He was also administered oral Albendazole (400 mg OD) over 4 weeks. The patient was completely asymptomatic at the 6 months and 1 year follow-up. Conclusion: Though rare, hydatid cyst should be considered a differential diagnosis for submandibular gland lesions. Hydatid cyst fluid (HCF) is highly antigenic and may cause allergic reactions ranging from mild hypersensitivity to severe anaphylaxis which may be potentially fatal for the patient. This may occur spontaneously, due to accidental trauma or iatrogenic manipulation. Hence, great precision and precaution need to be exercised during the surgical excision to prevent any spillage of contents in the surgical field. Clinical significance: Being extremely rare in the head and neck region, hydatid cyst is liable to be misdiagnosed unless there is a high degree of suspicion. It is imperative that a hydatid cyst be preoperatively diagnosed and positively confirmed as any accidental or iatrogenic trauma may cause release of the HCF. Also, the intraoperative dissection needs to be meticulous and precise and arrangements should be made in anticipation of complications to ensure a favorable outcome for the patient.