To diagnose and surgically manage the case of Gorlin–Goltz syndrome.

Gorlin–Goltz syndrome is an inherited autosomal dominant syndrome with a high level of penetrance and variable expressiveness.

This is a rare case series of Gorlin–Goltz syndrome with familial history in brother and sister, who were incidentally diagnosed with syndrome following presentation with multiple odontogenic keratocysts, bifid ribs, bilamellar calcification of falx cerebri and palmar pits.

Thorough clinical and radiographic assessment is needed to evaluate involvement of other bones. Gorlin–Goltz syndrome mandates genetic counselling for all the other family members due to inherited tendency. Long-term follow-up is required to warrant no recurrence of keratocyst.

Management strategy involves diagnosis with criteria and prompt surgical treatment with genetic advice followed by multidisciplinary approach to defer the extent of the syndrome.