To investigate the clinical characteristics and treatment of acute cerebellar ataxia in children associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The clinical features, neuroimages, therapy and prognosis of a girl with acute cerebellar ataxia associated with SARS-CoV-2 infection who was hospitalized in Children′s Hospital of Fudan University at Xiamen were retrospectively analyzed and the related literature were summarized. The patient was a girl aged 4 years and 11 months. She was admitted due to cough for 2 weeks and fever for 3 days. On the 3rd day of admission, she was treated by intravenous human immunoglobulin injection with a total amount of 2g/kg (divided into 4 days) and with methylprednisolone 2mg/kg. After significant improvement, she was discharged from hospital with oral prednisone acetate at 1mg/kg/d. At 11 days of follow-up, the girl had returned to normal, and prednisone was stopped. Three articles and 3 children with acute cerebellar ataxia associated with SARS-CoV-2 infection were reported. The age of onset was 5 years old, 13 years old, and 15 years old, respectively. All 3 cases were males, and the onset was 1-2 weeks after SARS-CoV-2 infection. All of them had ataxia gait. Two patients treated with intravenous methylprednisolone, and they were recovered to normal within 2 months. Acute cerebellar ataxia caused by SARS-CoV-2 infection usually appears 1 to 2 weeks after infection, and the clinical manifestations were ataxic gait, which was mostly caused by post-infection immune response, and the prognosis was good.