The prognosis of primitive neuroectodermal tumors is related to a number of factors and varies from person to person. The 3-year overall survival rate has been reported to be about 17.7% in one study, respectively. Primitive neuroectodermal tumors (PNET) are highly malignant small round cell tumors of neuroectodermal origin, with low incidence, most common in children and adolescents, with multidirectional differentiation potential, aggressive growth, and easy to recur and metastasize. PNET is sensitive to radiotherapy, and the prognosis varies greatly. Depending on the site of disease, there are two types of PNET: central PNET (c PNET) and peripheral PNET (p PNET), with the former mainly located in the brain parenchyma and spinal cord, and the latter mainly originating from peripheral soft tissues and the musculoskeletal system. The prognosis of this disease is closely related to tumor location, stage and treatment plan. According to some studies, the overall median progression-free survival is 10 months, the median overall survival is 18 months, and the 1-, 2- and 3-year overall survival rates are 76.9%, 42.6% and 17.7% respectively. It is recommended to go to regular hospitals for treatment and follow the doctor’s instructions to avoid delaying the disease.