Case Report: A ten-year-old boy was referred by a pediatrician for an ocular evaluation. This was a case of known diabetes for two years while on insulin and the pediatrician was concerned about diabetic retinopathy as the patient complained of progressive visual decline over the past two years. The patient was born of a first-degree consanguineous marriage to a cousin, with a normal birth history. He was diagnosed with diabetes at the age of eight, while receiving insulin. The referral letter from the pediatrician as well as a recent HBA1C report were indicative of good glycemic control. Additionally, the document reported an unremarkable systemic examination (afebrile patient with normal CVS, intact CNS, clear chest, and soft abdomen). His general health was good. He entered my office with a normal gait. The patient was well oriented in time and place. Its spontaneous and better corrected visual acuity with a manifest refraction; +1.00DS/-0.25DC@180 OR, or 6/60 OR. He was unable to discern any of the color vision patches during Ishihara's color vision test. Confrontation visual fields allowing careful counting of fingers were restricted, but confrontation tests appeared unreliable because patient cooperation was low throughout the examination. Extraocular motilities were full in OU. Both pupils were also round, but not very sensitive to light and without RAPD. Intraocular pressures measured 12 mmHg OR. Examination revealed bilateral ocular adnexa and anterior segments within normal limits. The earlier chambers were silent and deep. Dilated fundus examination revealed both clear OUs of the vitreous body. Both of his optic nerve heads were pale, consistent with the clinical appearance of optic atrophy. The remainder of the fundus examination was unremarkable, including a well-defined fovea, healthy macules, ...... middle of paper ......24. Hilson JB, Merchant SN, Adams JC, Joseph JT. Wolfram syndrome: a clinicopathological correlation. Neuropathological acta. 2009;118(3):415-28. Published online 05/19/2009.25. GN, JL M. Retinal function in a unique syndrome of optic atrophy, juvenile diabetes mellitus, diabetes insipidus, sensorineural hearing loss, autonomic dysfunction, and hyperalaninuria. Invest in ophthalmology. 1972;11:617-24.26. Carson M, Slager U, Steinberg R. Concurrent onset of diabetes mellitus, diabetes insipidus, and optic atrophy in a sibling. Am I Say Child. 1977; 131:1382-5.27. Barrett T. Optic atrophy in Wolfram syndrome (DIDMOAD). Eye (London). 1997; 11 (part 6): 882 - 8.28. Lim MC, Thai AC. A Chinese family with Wolfram syndrome presenting with diabetic retinopathy and rapidly progressive renal failure. Annals of the Singapore Medical Academy. 1990;19(4):548-55. Electronic publication 1990/07/01.