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 History: The patient is a four year old white male with a long history of recurrent pyogenic infections. He also has a history of photophobia and mild developmental delay. He was in his usual state of health when he developed fevers and right sided weakness. MRI and CT scans revealed white matter lesions with enhancement. A brain biopsy revealed a lymphohistiocytic infiltrate. During this time, EBV titers revealed seroconversion.

Physical Examination: The physical exam revealed a slightly obese 4 year old boy with a light, creamy complexion. He has light blue eyes, very light blond hair (Figs. A,B), and no evidence of nystagmus. Opthalomologic exam revealed decreased retinal pigmentation.

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