Pilomyxoid astrocytoma ( PMA) is a newly identified low-grade glioma (LGG) that was previously classified as a pilocytic astrocytoma ( PA). The term PMA was added to the World Health Organization (WHO) classification in 2007. Histologically, PMA shows monophagic architecture and protoplasmic cells or Rosenthal fibers are rarely seen. PMA has predominant myxoid background with frequent angiocentric pattern. And calcification or eosinophilic granular bodies are not seen. Firstly, PMA was classified as grade II due to its more aggressive behavior than PA. However, recent studies have shown histological and genetic overlap between PMA and PA, with some of PMA maturing into PA over time and less certainty that the PMA always follows a more aggressive course than PA. Thus, it is not clear that PMA should be assigned to grade II and the suggestion was made to suppress grading of PMA until further studies clarify their behaviors. Although PMA is generally a tumor of early childhood as a rare type of LGG, the Korean pediatric PMA case was not reported yet. Here we report 2 cases of PMA, the patients were 4 and 6 years old, respectively. They showed typical PMA features and now periodically followed after chemotherapy. Case 1 A 4-year old girl was admitted to our hospital due to nystagmus over 1 month. In brain computed tomography (CT) and magnetic resonance imaging (MRI), the suprasellar tumor (36 X 42 X 33mm) was found. The stereotatic needle biopsy was done and PMA was proven. After ventriculoperitoneal shunt operation and CCG-9952A chemotherapy she is followed periodically by checking brain imaging for 15 months. Case 2 A 6-year old boy visited our hospital due to left side weakness. He had intermittent high fever and coughing firstly. After 2 weeks, left side weakness was started from his leg. After 5days, the weakness was spread to his upper extremity and he admitted our hospital for work up. In brain MRI there is high signal mass (28 X 17 mm) on right midbrain at T2WI and DWI view. The needle biopsy of the tumor identified PMA. After the chemotherapy (Ghim’s protocol), we found only hemorrhagic cystic lesion in his brain (18 X 11 X 11mm) and there was no interval change by following radiologic work up.