Eagle syndrome is a bone disease where elongation of the styloid process leads to throat and neck pain, and in severe cases neurovascular symptoms such as syncope and neuralgia. The pathophysiology of Eagle syndrome is poorly understood with various theories having been proposed how this elongation is caused. To better understand the pathophysiology, we performed an work-up in 6 patients presenting with Eagle syndrome. Patients mainly presented with pain on turning the neck (100%), foreign body sensation (67%), tension in the neck (67%) and dysphagia (50%). The typical length of the styloid process ranges from 25 to 30 millimeters, [18F]NaF (sodium fluoride) PET/CT showed elongated styloid processes of 52.1 ± 15.6 mm (mean ± SD with increased turnover at the base of one the styloid processes. The removed styloid processes were further examined by histology, micro-CT, quantitative backscatter electron imaging (qBEI), Fourier transform infrared spectroscopy (FTIR) and circularly polarized light (CPL) imaging. Histology revealed one case of a fractured styloid process healing through callus formation and one case of pseudarthrosis. Bone mineral density and mineralization measured in the micro-CT and qBEI analysis and was similar in the styloid processes compared to cortical bone samples derived from the mandibular bone of separate patients. Circular polarized light microscopy showed collagen orientation comparable to cortical bone with a distinct separation of collagen structure between the mineralized structure and the surrounding soft tissue with FTIR analysis demonstrating a typical composition of bone. This altogether suggests that the elongated styloid processes in Eagle syndrome are mature bone, capable of endochondral repair, possibly growing from the base of the process through endochondral ossification and not a form of secondary calcification of the stylohyoid ligament as previously postulated.