Purpose of Review <p>To review current literature regarding the incidence of congenital nasolacrimal duct obstruction among pediatric patients with a diagnosis of childhood glaucoma, and to describe the distinguishing signs and symptoms on presentation in our experience at an academic, tertiary center caring for children with glaucoma.</p> Recent Findings <p>Congenital nasolacrimal duct obstruction (CNLDO) is a common, relatively benign condition that affects 5–20% of children. In contrast, primary congenital glaucoma (PCG) is a rare but urgent, sight-threatening condition. The incidence of CNLDO in infants with PCG has not been described previously in the literature and has implications for diagnosis and swift referral to an ophthalmologist to prevent blindness in infancy.</p> Summary <p>The incidence of congenital nasolacrimal duct obstruction among a population of 208 patients with diagnoses of childhood glaucoma was 1.4%, significantly lower than the typically cited incidences of 6–20% in the general population (<i>p</i> = 0.0087). 0 cases of primary congenital glaucoma presented with mattering, while 71% presented with clear tearing. In no cases did mattering mask an underlying diagnosis of glaucoma. When considering a referral to ophthalmology, primary care providers should be counseled to consider more urgent referrals for tearing when the tearing is clear, especially if associated with photophobia or corneal haze.</p>