Recently, the Department of Otorhinolaryngology, Head and Neck Surgery of the Third Affiliated Hospital of Southern Medical University operated on a five-year-old child with congenital gill-slit-ear-kidney syndrome and successfully repaired the congenital ear leakage for the child. The patient, Xiaoxing, five years old, from Heyuan, was hospitalized in a hospital due to purulent meningitis three months ago. During the hospitalization, he was found to have fluid coming out of the left nasal cavity, and the attending doctor considered it to be cerebrospinal fluid leakage and treated him according to purulent meningitis. The condition was relieved more than a month later, but the child still had nasal leakage and the meningitis was still recurring. Half a month ago, Xiaoxing’s parents brought Xiaoxing to the Third Affiliated Hospital of the Southern Medical University for help. Tian Guangyong, director of the Department of Otorhinolaryngology, Head and Neck Surgery, made a comprehensive analysis of the child’s condition, and after repeated studies by experts in the department, they believed that the child should not have a simple nasal leakage, but probably suffered from a rare disease – gill slit-ear-kidney syndrome. After discussion, it was decided to perform tympanic membrane puncture to clarify the diagnosis. The biochemical secretion result of the secretion from the middle ear cavity of Xingxing’s left ear was still considered to be cerebrospinal fluid, and there was a constant flow of clear fluid from the left ear, and there was no more secretion from the nasal cavity, so it was determined to be caused by cerebrospinal fluid ear leakage, and it was considered to be related to the patient because of her repeated illnesses of viral meningitis and suppurative meningitis in the past. After diagnosis, Director Tian Guangyong performed cerebrospinal fluid ear leakage repair surgery for Xiaoxing, successfully plugging the congenital ear leakage of Xiaoxing, after two weeks of post-operative observation, the little guy recovered well, and can be discharged from the hospital in the near future. According to Director Tian Guangyong, gill slit-ear-renal syndrome is a rare autosomal dominant genetic disease with an incidence rate of 1/40,000, which is highly susceptible to misdiagnosis and mistreatment. Director Tian explained that, in general, the external auditory canal, in addition to some shed epithelium and a small amount of dry cerumen (earwax), most people are clean, if there is a constant flow of abnormal fluid out of the external auditory canal, which is collectively referred to as ear leakage. Ear leakage is often overlooked and misdiagnosed. If the patient’s tympanic membrane is intact and the cerebrospinal fluid enters the nasal cavity from the Eustachian tube, many people mistakenly think that it is simply a runny nose and ignore it, especially in children with a runny nose, which is often mistakenly thought of by parents as the common cold and delayed in seeking medical attention. For the gill slit-ear-renal syndrome that Xiaoxing suffers from, the effluent is cerebrospinal fluid, and the patient is susceptible to retrograde infection, resulting in life-threatening meningitis, which requires timely surgical intervention to repair the leakage.