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Occipital encephalocele is a rare congenital anomaly that is characterized by herniation of brain tissue through a defect in the skull. Because of their enormous size, they pose a surgical challenge. Occipital encephalocele is the commonest of all encephalocele; the management of encephalocele defects requires immediate surgical closure. The author reports a case of a five months old female baby who presented a progressively increasing swelling over the occipital region. This swelling was present since birth. Surgery was planned to reduce the size of the swelling as well as its contents. The sac was excised and reduced sufficiently enough to accommodate the healthy-looking brain tissue. This infant did well postoperatively.