Amyloidosis is a rare disease characterized by the accumulation of an insoluble protein called 'amyloid' in various soft tissues and organs. Primary localized amyloidosis involving the mediastinal and cervical lymph nodes is extremely rare. A 68-year-old man was hospitalized a few days ago for recent cerebral infarction and was diagnosed with primary localized amyloidosis manifesting as mediastinal and cervical lymphadenopathy with calcification. In this review, we report a case of primary localized amyloidosis involving the mediastinal and cervical lymph nodes along with its computed tomography findings. Amyloidosis should be considered as part of the differential diagnosis when the lymph nodes show low central attenuation and various types of calcification.