Massive Wormian bone at the cranial apex: identification, correction and outcome.

We present the case of a three-month old African American female who presented with abnormal head shape and alopecia areata in the overlying skin. The patient underwent a subtotal cranial vault reconstruction at ten months of age. At surgery, a 7cm by 8cm Wormian bone was found at the cranial apex involving the coronal and sagittal sutures, obliterating the anterior fontanelle. In its center was an epidermoid cyst projecting intracranially between the cerebral hemispheres, which was also safely removed. At this time she has reached her developmental milestones without deficiency at three years of age.