Retinal arterial macroaneurysms are a relatively rare clinical entity that occur most frequently in the elder female population. They are strongly associated with systemic hypertension. Variable clinical presentations may occur, which may often lead to misdiagnosis. Correct diagnosis is essential for proper follow-up care and potential treatment. Differential diagnosis includes Coats' disease, angiomatosis retinae, Leber's miliary aneurysm, diabetic retinopathy, ectopic neovascular membrane, and malignant melanoma. The importance of the differential diagnosis is exemplified in a case report of a macroaneurysm which, upon initial ophthalmoscopic presentation, was concealed by surrounding retinal hemorrhage. The clinical features, differential diagnosis, management, and treatment of retinal macroaneurysms are discussed.