To report a family with congenital bilateral lacrimal punctum and canalicular agenesis.

A 45 year old male referred to our clinic for chronic tearing of both eyes. In examination, his four puncti of four lids were occluded. At operating room punctoplasty was performed but lacrimal canaliculi found occluded at exploration in both eyes. The patient stated that several members of his family complained from tearing so they were invited for examination and similar problem was found in his father, brother and two daughters.

Congenital bilateral lacrimal punctal and canalicular agenesis is an autosomal dominant disease; there are few reports of such patients as family. In this family four lacrimal puncti and canaliculi were absent; this feature is unique to our study families. At the time of this writing, there are no reports of familial cases of the disease from Iran.

Congenital bilateral lacrimal punctum and canalicular agenesis should be considered as a cause of tearing. In case of congenital bilateral agenesis of lacrimal puncti and canaliculi, the patient's family should also be investigated in terms of this problem.