Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare primary headache syndrome. However, some cases of secondary SUNCT are attributed to underlying diseases such as demyelination. We herein report a case of SUNCT with progression to neuromyelitis optica spectrum disorder (NMOSD). A 43-year-old woman developed headaches; 6 weeks later, she developed bilateral visual loss and numbness on the left side of her body. She was ultimately diagnosed with NMOSD.