To report the diagnosis and treatment of a case of Turner syndrome with meningoencephalocele and cerebrospinal fluid otorrhea. The patient, female, 17 years old, was diagnosed with Turner syndrome at the age of 10 and was treated with recombinant growth hormone. In the fourth year of treatment, the hearing loss in the right ear began to appear repeatedly. It was considered as "secretory otitis media", and the tympanic membrane was punctured many times to draw fluid After treatment, the hearing can return to normal after fluid extraction. Myringotomy was performed in our hospital, and a lot of clear liquid flowed out, which was indicated by biochemical examination as cerebrospinal fluid. Temporal bone CT showed bone defects in the tympanic roof and soft tissue swelling. Mastoidotomy, meningeal tissue retraction and leak repair were performed. Hearing returned to normal after operation, and no cerebrospinal fluid otorrhea occurred. This case suggests that pediatricians and otolaryngologists should collaborate in the treatment and follow-up of patients with Turner syndrome, especially during growth hormone therapy, and consciously incorporate ear health monitoring into routine diagnosis and treatment.