Background: Clinical presentation of Lyme-associated uveitis is poorly described. We reported here a case series of seven patients with uveitis related to Lyme disease and a review of the literature. Methods: A retrospective study in our university hospital between 1 May 2003 and 31 July 2016 on 1006 uveitis patients and review of Pubmed library. Results: Seven patients (71.4% male, mean age = 53 (38–70)) were diagnosed with a Lyme-associated uveitis. All anatomical types of uveitis were found (four intermediate, three anterior, and three posterior uveitis); most were unilateral (n = 6; 85.7%), one granulomatous and two with synechiae. Peripheral retinal vasculitis was present in four patients. They all had a risk of exposure or extra-ophthalmological symptoms. Antibiotic and steroid treatment was rapidly effective in all patients. Four patients presented recurrences of uveitis, of whom two received a second antibiotic treatment, which is quite common in literature. Persistent or recurrence of symptoms can be explained by three hypotheses: (1) reinfection, (2) relapse of original infection, and (3) autoimmune reaction. Conclusion: Lyme-associated uveitis appears varied. Hyalitis and involvement of the posterior segment and retinal vasculitis seem to be rather frequent. Its prognosis is mainly good, even if inflammation can be resistant or recurring.