Conclusion
We describe the ninth individual presenting with hypoketotic hypoglycemic secondary to an activating AKT2 mutation causing autonomous activation of the downstream insulin signaling cascade. Our patient’s dysmorphic features, developmental delay, and hemihypertrophy provide additional evidence of a broad phenotypic spectrum. Due to the anticipated lifelong duration of treatment, safe and tolerable options are needed. Our initial success with WMHMS indicates it is a promising option, although additional research is needed to establish dosing.