Objective: To analyze the clinical characteristics and causes of misdiagnosis of sparganosis. Methods: A total of 52 cases with sparganosis were enrolled from August, 2006 through August, 2018 in Department of Infectious Diseases of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine. The epidemiological and clinical data were retrieved and causes of misdiagnosis were retrospectively analyzed. Result: The sparganosis was mostly found in middle aged man with an average age of 39.8 years and male to female ratio of 1.36∶1. The sparganosis was highly likely to be misdiagnosed and 57.7% of patients (30/52) had not be recognized upon first clinical consultation. Thirteen patients with the cerebral sparganosis had been misdiagnosed as ascerebral tumor (4/52), brain abscess(2/52), encephalitis granuloma(2/52), and epilepsy(5/52), while patients with extracranial sparganosis(17/52)were considered as pulmonary infection (7/52), pulmonary Tuberculosis(1/52), acute appendicitis(2/52), chronic gastritis combined with twelve fat intestinal ulcer 1/52), intestinal Crohn with obstruction (1/52), liver abscesss (1/52), splenic space-occupying lesion (1/52), rheumatoid arthritis(1/52), chronicmyelocytic leukemia(1 /52). Inquiry of clinical history revealed raw frogs or snakes eating were seen in most of patients(63.5%, 33/52）with eosinophilia as a common laboratory findings (53.8%, 28/52). Serum anti-sparganosis. antibody was detected in all the 52 patients and the diagnosis were all confirmed by pathological examination and identification of worm body. Most cases were cured (84.6%, 44/52), with either praziquantel alone (42.3%, 22/52) or combined with operation (42.3%, 22/52). Eight cases (15.4%) had the relapse. Conclusions: The clinical manifestations of sparganosis varies among patients and is often misdiagnosed. The proper epidemiological investigation is critical for differential diagnosis, particularly for patients with atypical presentation. The MRI imaging study and serum screening of antibody against parasite are essential for establishing the diagnosis.