Bandeira, A.Cunha, S.Álvares, S.Martins, E.2011-06-012011-06-012010Nascer e Crescer 2010; 19(1): 54-560872-0754http://hdl.handle.net/10400.16/667ABSTRACT A 13 years old boy presented an abnormal habitus since birth with generalized paucity of fat, muscular overdevelopment and hepatomegaly. Laboratory tests revealed increased triglycerides. Congenital generalised lipodystrophy was diagnosed.porGeneralized lipodystrophyBerardinelli-Seip syndromejournal article