Abstract Background Encephaloceles are herniation of brain parenchyma through the defect in the dura and the skull bones. This case report reveals a rare association of frontonasal encephalocele, subependymal nodular heterotopias and cerebellar dysplasia with review of literature. Case presentation Frontonasal encephalocele is a rare entity. We report an 18-months-old male child who presented to the department of pediatrics with chief complaint of congenital swelling in the region of forehead. Swelling was increasing in size as told by the parents of the child. The child was born full term with normal vaginal delivery. There was no history of any obstetrical complications. The swelling was soft, transilluminant and pulsatile in nature. He was referred to the department of radio diagnosis for imaging evaluation. His CT and MRI images revealed defect in the anterior cranial fossa with herniation of meninges and brain parenchyma through it. There was also evidence of subependymal nodular heterotopias in this patient along with hypoplasia of left cerebellar hemisphere and left middle cerebellar peduncle with left cerebellar dysplasia. Conclusions This case report describes the coexistence of frontonasal encephaloceles, subependymal nodular heterotopias and cerebellar dysplasia, which is a very rare association.
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