Meckel-Gruber syndrome, which is firstly described by Friedrich Meckel and Georg B.
Gruber, is an autosomal recessive disorder that is characterized triad of occipital
encephalocele, bilateral renal dysplasia and polydactyly. Because of the genetic
heterogeneity, multiple organs can be affected. The worldwide incidence varies from 1 in
13,250 to 1 in 140,000 live births. We have reported a male baby born at 37-week gestation,
who has occipital encephalocele, polydactyly and cystic dysplasia of both kidneys. As a …

Meckel Gruber syndrome is an autosomal recessive disorder, characterized by a
combination of renal cysts or cystic renal dysplasia, developmental anomalies of the central
nervous system, hepatic dysgenesis and polydactyly. It is a rare syndrome with highest
incidence in Gujarati Indians and Finnish population. We report a case of Meckel Gruber
syndrome in non Gujarati Indian which was diagnosed on fetal autopsy.