Mandibuloacral dysplasia type A [MADA; Online Mendelian Inheritance in Man (OMIM) no. 248370] is a rare and complex laminopathy characterized by postnatal growth retardation, craniofacial anomalies, bone resorption at specific sites including clavicles, phalanges and mandibula, mottled cutaneous pigmentation, partial lipodystrophy (type A pattern), and insulin resistance (Novelli et al. 2002). The gene discussed is INS; the disease is mandibuloacral dysplasia with type A lipodystrophy.