Purpose To evaluate patients with lacrimal dysgenesis. Design Retrospective, n onrandomized, comparative case series. Participants Fifty patients with lacrimal dysgenesismanaged between 1992 and 2003. Testing/intervention The diagnosis of lacrimal outflow dysgenesis was made based on the following criteria: absent or hypoplastic punctum, canaliculus, lacrimal sac, and nasolacrimal duct, appearing in isolation or combination. Our management algorithmincluded observation, punc toplasty with intubation, or open lacrimal surgery, with or without intubation. Main outcome measure Success, partial success, or failure of treatment. Results Lacrimal dysgenesis in our 50 patients (23 male and 27 female) involved 83 eyes, distributed as follows: proximal in 74 eyes (89%), distal in 27 (33%), and bo th in 18 (22%). Thirty (60%) patients presented with isolated lacrimal dysgene sis, and 20 (40%) presented with a systemic syndrome or dysmorphism. Thirty th ree (66%) patients had bilateral involvement. Epiphora was the most common pres enting symptom. Eighteen (36%) patients had a positive family history. Open lac rimal surgery was performed in a total of 29 (35%) of the 83 eyes: 25 had succe ss, 3 had partial success, and 1 had failure. Conclusion Both sporadic and hered itary forms of lacrimal outflow dysgenesis may present as an isolated finding or a part of a systemic syndrome or dysmorphism, occurring usually with bilateral involvement and presenting at a younger age in the setting of systemic anomalies . Proximal and distal lacrimal outflow systems may be involved with epiphora as the most common presenting symptom. The algorithm we have presented provides a s ystematic approach to the management of lacrimal outflow dysgenesis. Purpose To evaluate patients with lacrimal dysgenesis. Design Retrospective, n onrandomized, comparative case series. Participants Fifty patients with lacrimal dysgenesismanaged between 1992 and 2003. Testing / intervention The diagnosis of lacrimal outflow dysgenesis was made based on the following criteria: absent or hypoplastic punctum , canaliculus, lacrimal sac, and nasolacrimal duct, appearing in isolation or combination. Our management algorithmincluded observation, punc toplasty with intubation, or open lacrimal surgery, with or without intubation. Main outcome measure Success, partial success, or failure of treatment. The involvement of 83 eyes, distributed as follows: proximal in 74 eyes (89%), distal in 27 (33%), and bo th in 18 (22% 60%) patients presented with isolated lacrimal dysgene sis, and 20 (40%) presented with a systemic syndrome or dysmorphism. Thirty th ree (66%) patients had bilateral involvemen Epiphora was the most common pres enting symptom. Eighteen (36%) patients had a positive family history. Open lac rimal surgery was performed in a total of 29 (35%) of the 83 eyes: 25 had succe ss, 3 had Conclusion Both sporadic and hered itary forms of lacrimal outflow dysgenesis may present as isolated finding or a part of a systemic syndrome or dysmorphism, occurring usually with bilateral involvement and presenting at a younger age in the setting of systemic anomalies. Proximal and distal lacrimal outflow systems may be involved with epiphora as the most common presenting symptom. The algorithm we have submitted provides as ystematic approach to the management of lacrimal outflow dysgenesis.