Microcephalic primordial dwarfism is a unique and rare group of skeletal dysplasias associated with characteristic facial features, microcephaly, and dwarfism. Of the many types of microcephalic primordial dwarfism, the most common is microcephalic osteodysplastic primordial dwarfism type II. It is associated with cerebrovascular aneurysms, moyamoya vasculopathy, and skeletal dysplasia. In addition, insulin resistance, hypertension, and hypodontia are common associations. These patients have relatively small body size and weight and, consequently, smaller than expected tracheal size with a potentially difficult airway. We describe the multisystemic details of microcephalic primordial dwarfism and our experience anesthetizing these individuals with many challenging complexities.