PurposeEpiphora in childhood is a frequent symptom that is typically associated with Congenital nasolacrimal duct obstruction (CNLDO). Nevertheless, inflammatory pathologies of the ocular surface as well as inside the eye, or even congenital glaucoma, must be considered in the differential diagnosis.MethodsA comprehensive literature review concerning CNLDO was conducted. Different therapeutic steps are categorized and summarized in order to reflect the existing staged therapeutic concept.ResultsFor CNLDO, a staged therapeutic concept is applicable, resulting in a cure rate of approximately 95% with only conservative or minimally invasive intervention. This concept includes five steps that encompass therapeutic interventions with increasing complexity. It includes conservative techniques, followed by probing and syringing, transcanalicular approaches without or with lacrimal intubation, and dacryocystorhinostomy which is the ultima ratio.ConclusionT o preserve the topographic anatomy as much as possible, therapeutic recommendations enable stepwise and individualized management of children with CNLDO