Background: Juvenile temporal arteritis (JTA) is a rare manifestation of TA before 50-year- old. Objective: To describe a patient with juvenile temporal arteritis (JTA) and ocular involvement and excellent response to temporal excision. Case report: A 41-year-old male patient with an unmarked past medical history pain in his right eye with no change in visual acuity and sensitivity on the right cephalic temporal area. An angio-magnetic resonance imaging revealed a hyper signal on the right ophthalmic artery compatible with retinal vasculitis. Prednisone 40mg/day for 3 months was started, and no change in pain was noted. Laboratory tests were normal, except for antinuclear antibodies positive in a titer of 1:160 and a nuclear dense fine speckled pattern, with positive anti-LEDGF75 antibodies. A diagnosis of JTA was determined. A temporal artery was performed, with normal histology. After surgery, all symptoms disappeared. Conclusion: This article describes an additional JTA case who was successfully treated after temporal artery biopsy/excision.