Faqeih et al. (2008) described a 2-year old male with micrognathia, aglossia and situs inversus with other deformities from Saudi Arabia. He was born to non consanguineous parents and had four siblings. At birth, he presented with a small oral cavity and tongue. At the age of 10-months, his weight, length, and head circumference were below the 3rd percentiles. He had a prominent metopic suture, long and smooth philtrum, small mandible and severe micrognathia. He had large and low-set ears, small mouth and oral cavity, high and narrow palatal arch and a rudimentary tongue. The lower alveolar ridge was prominent. He had right-sided apex beat, but there were no murmurs. He was hypotonic with normal reflexes, and had hypoplastic mandible, situs inversus totalis, and polysplenia.