IGF1 and Hypoglycemia: Our patient presented with novel features, including episodes of ketotic hypoglycemia with high anion gap metabolic acidosis, GHI, persistently low IGF1 levels, malar hypoplasia, hyperlaxity of the upper limbs, clinodactyly of the fifth fingers bilaterally, a single palmar crease on the right hand, shortening of the third toes bilaterally, and severe hypoplasia of the left third toe.