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Melkersson Rosenthal Syndrome is a rare neuro-mucocutaneous disorder characterized by the classic triad of facial swelling, recurrent facial nerve palsy and fissured tongue. The clinical course is usually progressive, and etiology is unknown. The authors describe oligosymptomatic Melkersson Rosenthal Syndrome in a young girl presenting sequentially with recurrent, metachronous facial nerve palsy and hemifacial swelling in early childhood followed by fissuring in the tongue in late-childhood. Histopathological examination from the affected labial area showed non-granulomatous inflammation. Bilateral facial nerve conduction and blink reflex studies showed asymmetrical affection of both facial nerves with mixed features of axonal and demyelinating involvement. The patient remained steroid-refractory, and subsequent attacks remitted with partial recovery. The combination of facial edema and facial palsy in a child should alert the physicians to the diagnosis of Melkersson Rosenthal Syndrome. A diagnostic mucosal biopsy, evaluation for systemic and oro-facial granulomatous disorders, and short course of corticosteroid treatment are recommended.

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Schimmelpenning-Feuerstein-Mims syndrome is a congenital neurocutaneous disorder, comprising of organoid epidermal nevus with a broad spectrum of multiorgan dysfunction (neurologic, skeletal, cardiovascular, ophthalmic, and urologic) secondary to postzygotic mutation in the early embryonic period. Predominant neurological manifestations include epilepsy, intellectual impairment, and focal deficits. Here, we report a 3-year-old girl who presented with epileptic spasms and had a characteristic linear sebaceous nevus. This report not only highlights the importance of early diagnosis of this condition but also emphasizes the need for multiorgan screening in children with seizures and nevi.