Pronounced acro-osteolysis is a signature symptom of MAD.10,12 Typical MADA is caused by the p.R527H mutation in the LMNA gene, while other LMNA mutations are responsible for different phenotypes.8 Regarding the phenotype, patients with MADA usually have distinguished features compared to MADB, which include more severity of clinical phenotype, early onset, renal disease, calcified skin nodules, premature birth and lack of acanthosis nigricans in the MADB type.4,10,12 Thus, a milder phenotype and slower disease progression is generally expected in MADA. Here, LMNA is linked to mandibuloacral dysplasia.