Parry-Romberg syndrome is a rare disorder with progressive hemifacial atrophy of unknown etiology. We reported 2 cases of progressive hemifacial atrophy with different neurological manifestations from Kuwait. The first case was a 14-year-old boy who initially presented with recurrent transient stroke-like episodes followed by focal seizures and hemifacial atrophy. Magnetic resonance imaging showed significant white matter changes and cerebral hemiatrophy. The second case was a 7-year-old girl who presented with complex partial seizures and hemifacial atrophy, her magnetic resonance imaging scan showed minimal changes in the hemiatrophy of the temporal cerebral lobe. Both patients' disease activity was well controlled with immunosuppressive therapy and anticonvulsants. Parry-Romberg syndrome should be considered in any child with unexplained neurological symptoms.