Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is characterized by pustular skin lesions and aseptic osteoarticular lesions. 1 The recognition of this syndrome by neurologists is important because it rarely induces skull lesion that may result in unexplained headache.Here, we describe a case of chronic headache with SAPHO syndrome. We further review previous cases and highlight a specific symptom of the headache that can be attributed to SAPHO syndrome. | C A S E REP ORTA 50-year-old woman with a 10-year history of palmoplantar pustulosis (PPP) presented with an occipital headache. Five years prior, she had idiopathic cervical spondylitis with severe neck pain. The bacterial culture of the vertebral tissue was negative, and she was treated with nonsteroidal anti-inflammatory drugs (NSAIDs); however, there was a sequela of dull pain. At 5 months before admission, she developed an occipital headache with tenderness. Although her personal doctor diagnosed her with neuralgia and prescribed NSAIDs, her symptoms persisted.On admission, she had red pustules on the palms and soles (Figure 1A). A slight bulge with tenderness was observed in the right occipital region. The culture tests of blood and cerebrospinal fluid were negative. Brain magnetic resonance imaging (MRI) with gadolinium (Gd) administration showed enhanced swelling in the subcutaneous and muscular tissues of the cephalic region that corresponded to the bulging locus (Figure 1B-a, b). Technetium-99m ( 99m Tc)-hydroxymethylene diphosphonate scintigraphy (Figure 1C) demonstrated increased uptake in the parieto-occipital bone, sternoclavicular joints with "bullhead sign," and the fifth cervical