This case illustrates the typical extracerebral manifestations of Parry Romberg syndrome with pronounced hemifacial atrophy in addition to less common intracranial findings.

The parenchymal enhancement seen in the presented case has not been described in the setting of Parry Romberg syndrome; however, the supra and infratentorial white matter signal abnormalities and microhemorrhages or calcifications are in keeping with previously reported findings.

Our patient underwent a brain biopsy which yielded non-specific findings of a lymphoplasmacytic infiltrate. The enhancement and parenchymal signal changes have remained stable over a 3 year follow-up period.