Microcephalic osteodysplastic primordial dwarfism, type II (MOPD II) is an autosomal recessive disorder characterized by prenatal growth retardation, microcephaly, skeletal dysplasia, short stature, and neurologic abnormalities. Dysmorphic facial features observed in MOPD II patients include: a prominent nose, slanting of the palpebral fissure, mildly dysplastic ears, and abnormal dentition. Patients often develop far-sightedness, scoliosis, unusual pigmentation, truncal obesity, and type 2 diabetes with time and their life expectancy is lowered due to a high risk of cerebrovascular anomalies. MOPD II is linked to mutations in the PCNT gene.