An infant presenting with an occipital encephalocele containing an epidermoid cyst: a rare case report

• Encephaloceles are neural tube defect where a protrusion of the brain or both meninges out of the cranial cavity through a bone defect. • In South-Asia, occipital encephalocele is less common than the anterior one. • Epidermoid cyst containing keratin, cellular debris, cholesterol, occasionally hair usually arises from ectoderm trapped within or displaced into the CNS. Encephalocele is a congenital neural tube defect characterized by the herniation of intracranial contents through a defect in the skull bone. An epidermoid cyst is an ectodermal cell-containing lesion in an abnormal location. We present a case of an infant who has been exhibiting a midline occipital swelling since birth. Clinical and radiological evaluations confirmed the presence of an occipital encephalocele. During surgery, the sac contained an epidermoid cyst, in addition to necrosed brain tissue and cerebrospinal fluid. The cyst was excised entirely, and the encephalocele was repaired. The postoperative period was uneventful, and the infant recovered well. An epidermoid cyst arising within an occipital encephalocele is a rare congenital anomaly. As far as we know, this is the first documentation of such an association. This article details the comprehensive management of this unique case, including the presentation, diagnostic assessments, surgical interventions, and additional supportive care measures