Abstract: Objective To analyze the clinical features of primary systemic amyloidosis in order to improve its diagnosis level. Methods Clinical features of 25 patients with primary systemic amyloidosis admitted to our hospital since 1991 were retrospectively analyzed. Results The median time from the occurrence of symptoms to the diagnosis of the disease was about one year. The main clinical symptoms were found to be weight loss, edema, short breath and acratia, fluster and chest distress, extremity numbness, abdominal distension, megaloglossia in 60%, 56%, 52%, 24%, 20%, 16% and 12% of patients, respectively. The principal signs including edema of low extremity, hepatomegaly, splenomegaly, hypertrophy of tongue were observed in 80%, 36%, 20%, and 20% of the patients, respectively. Injuries of organs, including the heart, liver, and kidney, as well as changes in routine blood test, were found in most of the patients. The misdiagnosed rate for nephritic syndrome, hypertrophy, cardiomyopathy, dilated cardiomyopathy, or other diseases, was as high as 64%. Conclusion Since the heart, kidney, liver and nervous system are the most commonly involved organs, amyloidosis should be considered if any clinical manifestations of hydremic nephritis, cardiac and hepatic disease, and abnormalities of the nervous system are found.