ObjectiveTo summarize regional and body distributions, diagnosis, treatment, and prognosis of echinococcosis outside liver in China. MethodsThe published literatures about echinococcosis outside liver in China (not including Hong Kong, Macao and Taiwan regions) from 2000 to 2015 were searched in the databases of CNKI and Wanfang. Data were extracted by using a standardized form and a retrospective clinical analysis was performed. ResultsA total of 66 published literatures about echinococcosis outside liver and data of 884 cases reported were collected. The regional proportions of cases reported were different, five high regions were Xinjiang (68.73%), Tibet (8.77%), Ningxia (6.75%), Qinghai (6.41%), Gansu (5.62%). The organ distributions of cases reported were also different, five high organs were lung (61.09%), bone (9.95%), brain (9.73%), spleen (8.60%), and kidney (6.22%). The main clinical symptoms of patients with echinococcosis outside liver included general asymptoms and organ dysfunction, and the most common examinations were X-ray (30.77%), B ultrasound (7.47%),CT(17.43%), MR (1.59%), and laboratory (36.31%). The main therapy choice was operation (73.30%), and some patients were performed operation combined with drug therapy. ConclusionRegional distribution of echinococcosis outside liver is almost the same as total echinococcosis, and distributed more in organs rich in blood supply, its clinical diagnosis mainly depends on imaging and immunology examination, and operation is still the main therapy choice.