Objective To investigate the clinical features and surgical treatment of spontaneous lateral sphenoid sinus recess cerebrospinal fluid rhinorrhea. Methods The clinical data of 22 patients with spontaneous lateral sphenoid sinus recess cerebrospinal fluid rhinorrhea from August 2003 to August 2014 were analyzed retrospectively. Nineteen of 22 patients underwent surgical repair via nasal and pterygoid process approaches under the endoscope and 3 underwent craniotomy repair. Results Repair surgery succeeded once in 20 patients. After endoscopic and craniotomy repair, 2 patients relapsed (2 months and 3 days after repair) and they were repaired again successfully. After procedure, 9 patients had elevated intracranial pressure (verified by lumbar puncture). Of the 19 cases with endoscopic repair, 3 experienced postoperative intracranial infection; 2 had postoperative hemorrhage and they were cure with conservative treatment; 2 had facial numbness, and they relieved gradually after 3 months. The patients were followed up for 2 to 122 months (mean 36.3 months) and no recurrence was observed. Conclusions Spontaneous lateral sphenoid sinus recess cerebrospinal fluid rhinorrhea is a rare disease which has typical characteristics and needs to be treated by surgery. Surgical repair via pterygoid process approach under the endoscope is a minimally invasive, safe, and effective treatment modality. Key words: Rupture, spontaneous; Cerebrospinal fluid rhinorrhea; Sphenoid sinus; Neurosurgical procedures; Neuroendoscopes