Congenital pyriform fistula is a rare disease of cervical gill origin caused by abnormal penetration or incomplete closure of the gill sulcus and pharyngeal sac during embryonic development, including congenital malformations of the third and fourth gill slits. Incidence: congenital pear-shaped fistula accounts for only 3-10% of all gill fissure malformations. Age of onset: More than 80% of patients develop the disease in childhood. Sex: There is generally no difference, but there is also a tendency for females to have a higher incidence. Site of onset: More than 90% of cases are located on the left side of the neck, which may be related to the asymmetric development of the fourth gill arch bilaterally in the embryo. Clinical manifestations: newborns and infants often present with a large cyst in the neck, which compresses the airway and leads to respiratory distress; children and adolescents, often with acute onset and a history of upper sensation, often present with an abscess on one side of the neck or acute suppurative thyroiditis, accompanied by painful or difficult swallowing; adults often present with a persistent fistula in the neck or recurrent cervical cellulitis, etc. Typology: includes cystic, sinus and fistula types, the latter two being common, and the fistula type is often the result of secondary infection ulceration or medically induced incision and drainage. Examination and diagnosis: Support laryngoscopy (or esophagoscopy) to detect an internal fistula is the gold standard for the diagnosis of CPSF. MRI and CT facilitate the determination of fistula alignment, adjacency and guidance of surgery. The combination of multiple tests can increase the positive diagnostic rate. Differential diagnosis: The clinical manifestations of CPSF, although typical, are not specific. Child and adolescent patients need to be differentiated from second gill fistula, thyroglossal fistula, epidermoid cyst infection, tuberculous fistula of the neck, and septic thyroiditis; infants and children need to be differentiated from lymphadenoma and cystic teratoma; adults need to be differentiated from cellulitis, cyst of the neck, and malignant tumors of the thyroid gland. Treatment: In the inflammatory phase, puncture or incision and drainage are preferred when abscesses are formed, and drugs are administered according to bacterial culture and drug sensitivity results; or antibiotics are used empirically. In the stationary phase, complete surgery is the key to eradication. Gastric tube nasal feeding should be routinely left in place after surgery to reduce hypopharyngeal infection and promote healing of the internal fistula in the pyriform fossa. The surgical approach includes: open surgery and endoscopic cautery. The open procedure is the traditional procedure and is now generally used for recurrent cases. The advantages of endoscopic cautery are that it is less invasive, has fewer complications, and has a recurrence rate no higher than that of the traditional procedure. It is preferred for primary cases, young patients, and sinus tract type.