Circumscribed choroidal hemangioma (CCH) is a rare congenital ocular tumor type that is usually benign and asymptomatic. CCH has only been reported once previously in the literature in association with a visceral neoplasm. Here, we present a newly described association between CCH and hepatic hemangioma (HH). We report the case of a 57-year-old woman diagnosed with asymptomatic HHs who presented with a 2- to 3-month history of central scotoma and blurred vision in her left eye. Assessment identified an orange-red elevated lesion with a central serous retinal detachment and subretinal edema. Complementary tests suggested a CCH. To our knowledge, CCH has only been reported once previously in association with a visceral neoplasm, making this newly described association between CCH and HH of clinical relevance. Ophthalmologists should be aware of the possible association between CCH and other visceral tumors as this association offers an opportunity for the early detection of this pathology.