We report the case of a 1-year-old child who presented with complaints of redness and defective vision since birth. The child had no systemic abnormalities. On examination, complete keratinization of the bulbar conjunctiva and cornea was noted with dry, lustreless and irregular surface. Corneal sensation was intact. Orbital MRI revealed bilateral agenesis of the lacrimal gland with normal salivary glands. The child was given vitamin supplementation, cyclosporine eye drops along with lubricants and tape tarsorrhaphy in the night. Permanent occlusion of both the lower puncta was done. There was decreased ocular surface congestion, with frequent wetting of the ocular surface, which continued in the months to follow. We present a case of isolated bilateral lacrimal gland agenesis with normal salivary glands, a rare cause of dry eye in children. An early diagnosis and conservative management can help in maintaining functional vision in such cases.