We experienced a case of crystalline retinopathy with crystalline deposits in the cornea and the limbal conjunctiva, and performed histological studies of the cornea, conjunctiva and peripheral blood cells. The patient was a 45-year-old man who had no abnormality in hematological studies and urinalysis. In the fundus diffuse atrophy of the retinal pigment epithelium and patchy foci of choroidal atrophy were observed, and sparkling yellowish-white spots were scattered, chiefly in the posterior region. Fluorescein angiography showed neither hyperfluorescence nor hypofluorescence at these spots. In the marginal cornea and the limbal conjunctiva crystalline deposits were observed. Histological findings by light microscopy and electron microscopy revealed sparse degeneration of epithelial cells in both the cornea and the limbal conjunctiva. But no substances corresponding to crystalline deposits could be identified. Inclusion bodies were detected including clefts in circulating lymphocytes and eosinophils. The inclusion bodies suggest that crystalline retinopathy results from a systemic metabolic abnormality.