There have been nine previously reported cases of intraocular lacrimal gland choristoma. This case report is of an infant with an intraocular lacrimal gland choristoma which was managed conservatively for a 19-month period until the onset of glaucoma. Tumour biopsy was initially performed because until this time the tumour's behaviour suggested it was not malignant. The latter was confirmed on biopsy, however hypotony resulted following the surgical intervention.