OBJECTIVE To describe a case of venous thrombosis secondary to unilateral racemose hemangioma (RH). METHODS Biomicroscopic examination, fluorescein angiography, and optical coherence tomography were used. The patient was a 12-year-old girl who was seen for progressive loss of visual acuity in the left eye. RESULTS Examination revealed venous thrombosis in the superior temporal vein area in the context of arteriovenous malformation (AVM). Treatment with intravitreal triamcinolone (4 mg) resulted in anatomic and functional improvement. CONCLUSIONS This was an exceptional case of branch retinal vein occlusion secondary to RH in a girl without known risk factors for venous obstruction. The obstruction was attributable to the large-caliber superior temporal artery, which, on forming a loop in the region of the venous branch, produced an obstruction of venous branch blood flow.