A 4-month-old boy was admitted to the hospital after his parents noticed fog in his right eye for 27 days. Physical examination revealed multiple light brown macules on the skin. The intraocular pressure (IOP) of the right eye was 34.4 mmHg (1 mmHg=0.133kPa) and the corneal was enlarged and edema while the aqueous humor was cloudy with blood in the right eye. Yellow peripheral anterior synechia was seen on the temporal and inferior iris. Ultrasound biomicroscopy (UBM) showed extensive adhesion closure of the anterior chamber angle and the local thicken iris with the nodule. No obvious abnormality was observed in the left eye. It was confirmed as juvenile xanthogranuloma by skin lesion biopsy. IOP was normal after systemic and local therapy with corticosteroids and local IOP lowering medication. Follow up 35 months, IOP is normal without medication.