Background: Only few reported cases of tectocerebellar dysraphia with occipital encephalocele have been reported in the literature.
Case presentation: Three month baby boy, the first child of healthy, consanguineous parents presented with a small swelling over the occipital region since birth. The child also used to have apneic spells without cyanosis and spontaneous recovery. CT scan showed absence of the cerebellar vermis, absence of tectum and the 4(th) ventricle communicating with the occipital menigocoele sac and an occipital bone defect. The excision of the encephalocoele sac was performed, however the child continued to have apneic spells and did not do well.
Conclusion: In our child irregular respiration probably was the manifestation of the tecto-cerebellar dysraphia syndrome complex and associated shunt malfunction followed by seizures decompensated the physiology of the child leading to fatal outcome.
Keywords: Dandy-Walker syndrome; Meningocele; Nevus; Pectus excavatum; melanocytic.