Abstract: Diffuse intrinsic pontine glioma (DIPG) is a highly invasive tumor located in the pons (middle) of the brain stem. They are usually diagnosed during childhood and account for 10%15% of primary brain tumors in children. DIPG has a very poor prognosis. Fewer than 10% of DIPG patients survive more than 2 years after diagnosis. The imaging manifestations of DIPG are typical, and biopsy is only performed in atypical cases. The tissue specimens of newly diagnosed DIPG are very few and limit its molecular biological research. Recent advances in surgical and molecularanalytic techniques have increased the safety of biopsy which has already been used in many clinical trials step by step. The research of DIPG's molecular pathogenesis and treatment is sure to achieve new breakthroughs.