We aimed in this report to discuss the embryology, anatomy, theories of ossification and symptoms, clinical presentation, and diagnosis of the stylohyoid chain (SHC) variations, together with the role of radiographs, computed tomography (CT) and three-dimensional (3D)-CT in showing these variations. Because CT/3D-CT additionally facilitates visualization of the entire SHC with different axes, it is the most valuable method for establishing the relationship between the SHC and the surrounding tissue. SHC variation can be discovered during CT performed for indications other than ossified SHC. It is important to diagnose whether or not the SHC is ossified, since one of the treatment procedures in ossified SHC is total excision. If the clinician and radiologist are aware of these variations observed in the SHC, patients with vague symptoms may be spared unnecessary investigations and may be properly diagnosed earlier.
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