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A 10-year-old Japanese girl presented with multiple, small, flat, dark brown clusters of retinal pigment epithelium in both eyes. She had no other symptoms. The appearance was consistent with congenital grouped pigmentation of the retina or bear track spots. A unique co-existing feature was the presence of small, albinotic spots within the entire retina. Fluorescein angiography demonstrated a persistent hypofluorescence correlating with the grouped pigmentation of the retina and granular hyperfluorescence correlating with the albinotic spots. Her mother also showed irregular pigmentation and albinotic spots in both eyes. We describe an extremely rare case of congenital grouped pigmentation and albinotic spots occurring in the retina of the same individual.

An Unusual Presentation of Concurrent Congenital Grouped Pigmentation of the Retina with Albinotic Spots