Lipodystrophies (LD) are a heterogeneous group of rare diseases characterized by a deficit of adipose tissue, decreased leptin levels, and metabolic abnormalities such as insulin resistence and diabetes. Lipodystrophies are classified as genetic or acquired and according to the distribution of fat to partial or generalized. Acquired generalized lipodystrophy (AGL) has a later onset (childhood or adolescence) compared with congenital LD and is more common in women than men.We present a case report of a 14-year-old girl suffered from autoimmune hepatitis (AIH) from the age of 3, anti smooth muscle antibody positive, histological examination confirmed chronic active hepatitis without cirrhosis. At the age of 12, she was admitted to the hospital for the development of generalized loss of subcutaneous fat, masculine habitus with prominence of subcutaneous veins on the limbs, signs of acanthosis nigricans were visible on the neck, in the axillae and around the navel. She had numerous tiny fibromes on her eyelids. On physical examination, her liver was 2 cm palpable below the costal margins The weight of the patient was 37 kg, BMI 16,4, height 150 cm. Her weight to height was at the 30th percentile.Bilirubin 9.9 umol/l, ALT 3.82 ukat/l, AST 2.37 ukat/l, GGT 0.94 ukat/l, total cholesterol 2.5 mmol/l, HDL 0.5 mmol/l, LDL 0.6mmol/l, triglyceride 3.13 mmol/l, C3 level 1.18 g/l, C4 level 0.09 g/l, IgG 21.4 g/l. Leptin level was 1.9 ug/l (n 4-11 ug/l). Fasting glycaemia was 6,4 mmol/l, HbA1C 47 mmol/mol. At the 120 minute oral glucose tolerance test, the glucose level was 8.8 mmol/l, C peptide 5628 pmol/l, insulin 268 mU/l which documents the state of insulin resistance and disorder of glucose tolerance. Positive anti mitochondrial, anti glutamic acid decarboxylase, anti zinc transporter 8 and anti granulocyte antibodies have been reported. The patient was shown to have a haplotype associated with type 1 diabetes (DRB1*03, DQB1*02, DQA1*05). Cardiological examination with finding hypertrophic cardiomyopathy. Hepatosplenomegaly was reported on ultrasonographic examination. Performed liver biopsy with steatofibrosis.The patient was started on diet therapy. She also required insulin treatment for AIH relapse and corticosteroid therapy.The autoimmune diseases associated with AGL include both organ-specific and systemic autoimmune diseases. A number of positive autoantibodies are reported in patients with AGL, but may not be associated with clinical manifestations of the disease. We consider that it is necessary to monitor these patients to see if the disease does not manifest itself in time.