Purpose To provide new insights into the pathophysiology, prevention and diagnosis of cerebral venous thrombosis (CVT) associated with iatrogenic cerebrospinal fluid (CSF) leaks and/or external CSF drainage. Methods Case report and literature review. Results We describe the case of a 30-year-old woman who developed a CSF fistula after lumbar spinal surgery. The treatment included rest, hydration, caffeine, and continuous lumbar CSF drainage. After closure of the fistula, the patient complained of severe orthostatic headache. Thrombosis involving the superior sagittal sinus, the right transverse sinus, the right sigmoid sinus, and the right jugular vein was diagnosed after neurological deterioration.