Parry Romberg syndrome (PRS) is an unusual neurological entity characterized by progressive hemifacial atrophy. We describe the case of a 17-year-old male who presented with recurrent seizures and facial asymmetry of 1 year duration. Computed tomography (CT) of the head showed right-sided hemifacial atrophy involving the subcutaneous fat, muscles, and bones alongwith subcortical white matter hypodensity, having parenchymal foci of calcification. The etiology of PRS is debated from being secondary to abnormalities in fat metabolism to autoimmune and inflammatory causes. The treatment is symptomatic and is targeted at relief of seizures and migraine. Steroids and other immunomodulators have proven successful in some cases. Surgical correction may be done once the disease stabilizes.