Bilateral agenesis of the lacrimal puncta and enlargement of the nasolacrimal canal are rare anatomic variants. The authors present 2 familial cases: a 39-year-old woman with bilateral dacryocystocele and lacrimal puncta agenesis and her 46-year-old brother, who had a long history of epiphora and recurrent dacryocystitis, and also had bilateral lacrimal puncta agenesis and a left dacryocystocoele. The authors report the endoscopic, CT, and MRI findings, and describe the surgical treatments, by endoscopic dacryocystorhinostomy in the first case, and conjunctivodacryocystorhinostomy with Jones tube in the second case. At 24 months after surgery, both patients' symptoms were improved.