Germline mutations of CEP55 in humans have been described in two lethal CEP55-associated syndromes, Meckel-Gruber syndrome (MKS)-like Syndrome [4,5] and MARCH (Multinucleated neurons, Anhydramnios, Renal dysplasia, cerebral hypoplasia, and Hydranencephaly) [6]. The gene discussed is CEP55; the disease is renal dysplasia.