Nevoid basal cell carcinoma syndrome (NBCCS) is rare in black persons,
We describe an 11-year-old black boy with NBCCS who presented with ex
otropia and a painful, expanding, cystic mass in the left posterior al
veolar ridge, Further examination revealed odontogenic keratocysts wit
h palmar and plantar pitting. Less than 5% of reported patients with N
BCCS are black. To our knowledge, this is the first report of a black
patient with NBCCS presenting with exotropia and an impacted molar dis
placed into the orbit by an odontogenic keratocyst.