Objective To explore the typical disease characteristics, key diagnostic points and treatment strategies of immunoglobulin light chain (AL) amyloidosis, so as to enhance understanding of the disease and its diagnosis and treatment. Methods The raccoon eye sign of a 63-year-old male patient with AL amyloidosis was reported, and the clinical manifestations of the patient were analyzed. A literature review was conducted by searching the PubMed database for relevant case reports, focusing on clinical features and prognosis of AL amyloidosis with periorbital purpura. Results A total of 15 patients with AL amyloidosis characterized by "raccoon eyes" were retrieved from the PubMed database, and together with the case reported by us, a total of 16 patients were included in the analysis. There were 8 males and 8 females with an average age of (63.7±7.2) years old for males and (67.8±8.2) years old for females. Of the 16 patients, 9 had kidney involvement and 8 had heart involvement. Renal involvement was manifested as proteinuria and/or impaired renal function. Cardiac involvement was primarily characterized by left ventricular hypertrophy and elevated levels of N-terminal pro brain natriuretic peptide. In addition, some patients presented with hepatic, pulmonary, and neurologic involvement and macroglossia. AL amyloidosis may also presented with nonspecific symptoms, mainly fatigue and unconscious weight loss. Five patients died within a few months after presenting with raccoon eye sign. Conclusion Clinicians should be alerted to the possibility of AL amyloidosis when patients present with the typical symptom of "raccoon eyes", and make a definitive diagnosis and treatment as soon as possible.