Introduction: The pathophysiology of reversible cerebral vasoconstriction syndrome remains incompletely understood. However, failure of cerebral autoregulation, sympathetic overactivity, disruption of the blood–brain barrier, and glymphatic system dysfunction play a central role.
Case report: We present the case of a 35-year-old woman with a history of cerebral vasoconstriction syndrome who developed new episodes of thunderclap headache and intermittent hypertension two years after the initial event. During hospitalization, non-invasive neuroimaging suggested a high probability of recurrent vasoconstriction syndrome. Due to persistent autonomic symptoms, further evaluation revealed a pheochromocytoma.
Conclusion: In patients with recurrent or atypical symptoms of reversible cerebral vasoconstriction syndrome, secondary and potentially treatable causes should always be considered.
