Cases of congenital disorders of glycosylation (CDGs) are rare, and the occurrence of hemorrhagic infarction is also rare. The etiology is unclear. A 3-year-old Asian boy with CDG type 1A was hospitalized with pneumonia. Consciousness disturbance and hemiplegia appeared after high fever and were associated with disseminated intravascular coagulation. Magnetic resonance (MR) images showed subcortical hemorrhagic infarction due to anterior superior sagittal sinus occlusion. Follow-up computed tomography revealed the enlargement of a right frontal lobe hematoma with a midline shift.