We present a case with giant arteriovenous aneurysmal malformation with different imaging modalities, including fundus photography, fundus fluorescein angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography. A 43-year-old Turkish female presented with gradual blurred vision in her left eye. Her best-corrected visual acuity was 20/200. The fundoscopic examination revealed hard exudates and microaneurysms around the macula. Spectral domain optical coherence tomography showed cystoid macular edema and serous macular detachment. Fundus fluorescein angiography and optical coherence tomography angiography showed bean-like arteriovenous anastomosis between the inferior venous vascular arcade and the superior arterial vascular arcade. An en face angiogram using optical coherence tomography angiography within the deep capillary plexus also showed venous collateral channels across the horizontal raphe. The patient was diagnosed with arteriovenous aneurysmal malformation in association with type 1 aneurysmal telangiectasia. Ophthalmologists should note that giant aneurysmal lesions can occur in type 1 aneurysmal telangiectasia patients.