Objective Spontaneous cerebral spinal fluid otorrhea (SCSFO) is rare ,the aim of this study is to analyze and explore the clinical characteristics, surgical methods and effect of SCSFO. Methods A retrospective analysis of the clinical data of 10 patients with SCSFO admitted to our department from 2015 to 2020, we summarized the clinical features, imaging features, surgical methods, perioperative management, and postoperative follow-up results. Results All the cases including 6 females and 4 males; 3 children and 7 adults; the history ranged from 25 days to more than 20 years; 9 cases were unilateral and 1 case bilateral. Among the 3 children, all were congenital severe sensorineural hearing loss, and all of them were diagnosed with rhinorrhea or meningitis; Among the 7 adult patients, 5 cases were suffered from ear fullness and hearing loss, of which 1 case was accompanied by otorrhea; 1 case was suffered from rhinorrhea; 1 case was suffered from ear pain accompany with meningitis;4 cases received myringotomy. Among the 3 children (4 ears), the leak points were all located at and around the stapedial foot plate. Among the 7 adults,2 patients had stapes deformity, the leaks points at the stapedial foot plate in 2 cases, tympanic canopy in 3 cases and mastoid canopy in 2 cases. Epilepsy and increased intracranial pressure occurred in 1 case after operation. The 10 patients were followed up for 5 months to 6 years, and there was no recurrence of cerebrospinal fluid otorrhea. Conclusion SCSFO is easy to be missed and misdiagnosed. Some patients are often repeatedly treated by rhinitis, meningitis, secretory otitis media in the early stage. This even led to severe intracranial infection. Therefore, it is particularly important to identify the early symptoms of SCSFO, surgical exploration is recommended to accurately search for leaks, and exact repair of leaks is the key.