We report the first case (to our knowledge) in the English-language literature of stroke syndrome associated with a widespread sebaceous nevus. A 25-year-old white woman presented with the sudden onset of left-sided weakness, dysarthria, and hypotension. She was diagnosed as having Bell palsy 12 days before presentation and was treated with prednisone. Her medical history was significant for a right ocular mass that had been resected when she was 5 years old. She had no history of seizures or cardiovascular, renal, or skeletal problems. Her family history was unremarkable. Her only daily medication was an oral contraceptive.