BACKGROUND: With increasing treatment options available, neuroendocrine tumor has become a chronic disease and may present later on with atypical manifestation of disease spread once resistant to treatment. CASE PRESENTATION: A 74-year-old white British woman undergoing treatment for metastatic well-differentiated neuroendocrine tumor for the past 9 years presented with a brief history of mild frontal headache, and progressive left ptosis and ocular palsy. She had no visual loss, and had neither speech nor motor deficit. At the outset, it was crucial to exclude acute or missed stroke. An urgent magnetic resonance imaging of her head revealed an unusual skull base metastasis extending into the cavernous sinus, with no peritumoral edema. Following discussion at a specialist neuro-oncology meeting and a neuroendocrine tumor multidisciplinary team meeting, she proceeded to have conventional fractionated radiotherapy followed by subsequent palliative chemotherapy. CONCLUSIONS: Intracranial metastasis is rare in patients with neuroendocrine tumor, particularly in those with well-differentiated histology; skull base metastasis is even more uncommon. Management of intracranial metastasis from a rare tumor should always be discussed in a specialist multidisciplinary meeting. Surgery or radiotherapy, including stereotactic radiosurgery, should be considered in skull base metastases. Hormonal abnormalities may occur following radiotherapy to skull base metastases and should be monitored closely in the first few months post treatment.