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Juvenile temporal arteritis (JTA) is a recently-described and little-known inflammatory disease and its etiology is undetermined. Less than forty cases have been published. This paper is aimed at reporting the largest JTA series and to compare it to literature data to better evaluate its characteristics at diagnosis, its evolution and treatment options. We conducted a retrospective and descriptive multicentric study in France by identifying adult patients under the age of 50 which had a pathological temporal artery biopsy owing to the presence of a temporal arteritis. Patients with temporal arteritis as a manifestation of systemic vasculitis were excluded. We included 12 patients and the literature review identified 32 cases described in 27 articles, thus a total of 44 patients – 34 men and 10 women – with a median age of 30 and a maximum of 44. All patients presented either a lump in the temporal region or prominent temporal arteries, and 47.7% of patients suffered from headaches. Only 11.4% of patients presented general symptoms and 6.8% a biological inflammatory syndrome; 34.1% had peripheral blood eosinophilia; 83.7% presented a single episode and complete excision without further treatment was documented for 72.7%. Pathology analysis revealed infiltrate of inflammatory cells in the arterial wall in 97.6% of patients but also sparse giant cells for 25% and granuloma for 22.9%, perivascular extension of the inflammation for 82.6%, and presence of lymphoid follicles or germinal centres for 60%. Clinical relapses were present in 16.3% of cases. JTA is a rare, localized and benign disease. The majority of cases have only one episode which is cured by local surgery. • Juvenile temporal arteritis, is a rare, localized, and benign disease affecting the temporal artery in patients aged less than <50 years. • JTA presenting as a lump in the forehead, sometimes painful, without systemic symptoms or biological inflammation. • JTA is most frequently cured by biopsy or excision. • Kimura disease and angiolymphoid hyperplasia with eosinophilia must be considered as alternate diagnosis. [ABSTRACT FROM AUTHOR]