Background: Lyme disease, caused by Borrelia burgdorferi, is a spirochetal disease. Lyme disease-related ocular findings may also provide important clues. Ocular involvement is most commonly seen as uveitis, chorioretinitis, conjunctivitis, keratitis, episcleritis, papillitis, panuveitis, ischemic optic neuropathy, papilledema, and retinal vasculitis. Case: A 27-year-old male patient was admitted with a history of fatigue, malaise, and sudden loss of vision in his left eye for 3 days. The best visual acuity was found 20/20 in the right eye and 20/400 in the left eye. Fluorescein fundus angiography showed no pathological findings in the right eye; but hyperfluorescence that was compatible with choroiditis foci was seen in the left eye. Optical coherence tomography (OCT) showed choroidal thickening in the left eye compared to the right eye. Lyme IgM antibody was found to be positive, explaining choroiditis etiology, while IgG values were found to be negative. Western blot verification test was positive. The patient was treated with 2 × 100 mg doxycycline (21 days) with a diagnosis of Lyme disease, prednol 1 mg/kg/day (10 days) for choroiditis. Omeprazole tablets were given 1 × 1 during the period of cortisone intake. On the third day of treatment, visual acuity increased to 20/200 and continued to increase until reaching 20/20 in the second week. Conclusions: Lyme disease is rare, but must be kept in mind when investigating the etiology of chorioretinitis and retinal vasculitis. The patient reported here is, to our knowledge, the second case reported in literature that shows atypical clinic for Lyme disease with unilateral chorioretinitis without Erythema chronicum migrans (ECM).