A 2-year-old healthy white boy had a vitreous hemorrhage in the left eye but no history or external signs of trauma. Computed tomographic and ultrasonographic examinations were not diagnostic. A pars plana vitrectomy performed when the patient was 2 1/2 years old revealed a posterior pole lesion diagnosed clinically as a combined retinal-retina pigment epithelial hamartoma. Such lesions may be symptomatic because of decreased vision, macular puckering, and strabismus; vitreous hemorrhage has not previously been reported as the presenting clinical sign or the predominant cause for reduced vision.