A three day-old male infant with Larsen's Syndrome is presented. The patient is presented with the typical facial appearance of this disease consisting of a flat nose, hypertelorism, a prominent forehead as well as multiple joint dislocations. A complete postmortem examination was carried out which revealed the added organ abnormality of a cystic kidney of Potter's type IV combined with double kidney pelves and double ureters. Various features of the disease are discussed with a brief review of the literature.