Retinal astrocytic hamartoma and retinoblastoma may be very similar clinically and their differentiation in atypical cases can be difficult, even with the use of ultrasonography and computed tomography. In such cases, a close follow-up is recommended before enucleation. This paper reviews the case of a 18 month old girl who presented with a solitary retinal astrocytoma of the right eye, without any other physical or ocular disorder. The initial presentation simulated a retinoblastoma; nevertheless atypical patterns as yellow calcifications and the lack of tortuous and dilated feeding blood vessels were present. Ophthalmoscopic and ultrasound regular evaluation did not reveal any change after one year follow-up. Additional investigations performed in order to exclude tuberous sclerosis (neurological and dermatological examination, CT-scans) showed no other organ involvement, which ruled out a phakomatosis. The clinical appearance and course of astrocytic hamartomas, its differential diagnosis from other retinal tumors, especially retinoblastoma, and its association with tuberous sclerosis are discussed.
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