Dysencephalia Splanchnocystica

Dysencephalia Splanchnocystica also called as Meckel-Gruber syndrome is an autosomal recessive disorder1. It is characterized by a traid of occipital encephalocele, large polycystic kidneys, and postaxial polydactyly2. The major diagnostic criteria of MGS include at least 2 of these 3 classic manifestations, occipital encephalocele, cystic renal dysplasia, and polydactyly found in 90 %, 100 % and 83.3 % respectively 3. It is a rare syndrome with highest incidence in Gujarati Indians and Finnish population. We are reporting a case of Dysencephalia Splanchnocystica in a non Gujarati Indian from a municipal town, Sirkazhi in Nagapattinam district in Tamil Nadu, India.