Spinal intradural primary malignant peripheral nerve sheath tumors (MPNST) are 
rare in patients without neurofibromatosis. Here we represent a 3-year-old girl 
of primary intradural spinal malignant peripheral nerve sheath tumor. The tumor 
was removed partially and MPNST was diagnosed in the histopathological 
examination. Her condition deteriorated due to acute hydrocephalus in the 
following days. In this article, we discuss the clinical presentation, imaging, 
treatment, and prognosis of our patient and the other 22 patients of primary 
intradural MPNST, found in the literature. The Kaplan?Meier method was applied 
for univariate analysis and Cox proportional hazards model for multivariate 
analysis. This analysis showed that age, was an important factor predicting 
short-term survival of patients with MPNST.