The authors present a unique case of bilateral isolated choroidal melanocytosis in an annular pattern that, to our knowledge, has not previously been described. A 44-year-old asymptomatic American Indian woman presented with bilateral choroidal hyperpigmentation in an annular pattern for 12 clock hours around the peripheral retina. No other uveal, scleral, or cutaneous hyperpigmentation was observed. Optical coherence tomography did not demonstrate choroidal thickening or subretinal fluid. The diagnosis of bilateral isolated choroidal melanocytosis was made. Long-term follow-up of this patient and other cases described in the literature is necessary to determine the malignant potential of these lesions. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e74-e76.].