To report the progression of a patient with retinal racemose hemangioma after 5 years of follow-up. Observational case report. A 14-year-old girl was diagnosed with an isolated retinal racemose hemangioma with retinal arteriovenous malformation in the posterior pole of the left eye fundus. At the fifth year of follow-up, a spontaneous enlargement of the previously normal vessel was found above the original lesion. The patient's vision remained stable, and no complication was observed. Retinal racemose hemangioma was once considered stable and nonprogressive, but progression and expansion of the lesion may also occur, so long-term follow-up is necessary.