Objective: To demonstrate the clinical characteristics and surgical effects of congenital lacrimal gland cutaneous fistula with ectopic lacrimal gland. Methods: Observational case study and literature review. The results of appearance photographs, lacrimal duct probing and irrigation, digital subtraction imaging of the lacrimal fistula, and CT scanning of lacrimal fistula in a patient diagnosed with lacrimal gland cutaneous fistula with ectopic lacrimal gland were recorded. The surgical treatment for the patient was explored. The surgical strategy entailed resecting the lower segment of the lacrimal gland fistula and ectopic lacrimal gland, combined with transposing the upper segment of lacrimal gland fistula for conjunctival sac anastomosis, instead of using lacrimal gland catheter. Pathological examination and postoperative follow-up were conducted. Results: Ophthalmic examination revealed a fistula with a diameter of approximatedly 1 mm in medial-temporal 1/3 of the upper eyelid of the left eye. The skin surrounding the fistula was sunken and covered with tufts of hair. There was a paroxysmal discharge of clear fluid from the fistula. Lacrimal examination showed that the opening of the fistula on the skin of the left upper eyelid was directed outward and upward. Fistula exploration was conducted through the opening. The fistula tract initially coursed medially and caudally, then laterally and caudally. At a depth of 3.5mm from the dermal surface, a soft resistance was encountered that could not be overcome with forced pressure. The irrigation fluid refluxed along its original pathway, with no pus or bloody fluid regurgination. Digital subtraction imaging of the lacrimal fistula in the left eye demonstrated that contrast media remained and formed fanshaped spaces in the depth of the fistula. CT results of lacrimal fistula revealed a high density of lacrimal gland in left eye. During outpatient follow-up six months after surgery, the incision on the left upper eyelid was observed to have healed well, with no recurrence of the fistula The transposed lacrimal fistula successfully diverted tears into the conjunctival sac of the upper fornix. Conclusions: Congenital lacrimal gland cutaneous fistula combined with congenital ectopic lacrimal gland and ectopic hair is rare in clinical practice. Detailed preoperative examination and accurate diagnosis are extremely beneficial for guiding treatment. When the main lacrimal gland is normal, a better treatment choice involves completely removing the ectopic lacrimal gland tissue and transferring the lacrimal gland fistula to the conjunctival sac of the upper fornix to replace the lacrimal gland catheter.