Hepaticglycogenosis is an inherited abnormality characterized by excessive glycogen accumulation in hepatocytes. It is a hepatic complication of poorly controlled type 1 diabetes mellitus. It combines hepatomegaly, growth failure, and hepatic cytolysis as well ascushing-like feature. Hepatic glycogenosis is only diagnosed by liver biopsy. We report the observation of three clinical cases: N°1: A 16-year-old male, with type 1 diabetes mellitus since a year. Admitted for keto-acidosic decompensation, he presented dyspnea, a distended abdomen with painless hepatomegaly. Abdominal ultrasound showed 21 cm homogeneous hepatomegaly. The anatomo-pathological study of the liver biopsy confirmed the diagnosis of hepatic glycogenosis.N°2:An 18-year-old female, with type 1 diabetes mellitusfor 14 years with a severe growth failure, she presented a major cytolysis with hepatic cholestasis for 5 years. Abdominal ultrasound showed homogeneous hepatomegaly.Theanatomo-pathological study of the liver biopsy confirmed the diagnosis of hepatic glycogenosis.N°3:A 19-year-old female, with type 1 diabetes mellitusfor 8 years, epilepsy and delayed puberty. The biological check-up had objectified a very significant cytolysis and major dyslipidemia. Abdominal ultrasound shows hepatomegaly associated with hepatic steatosis. A liver biopsy was indicated, but not performed as the patient was lost to follow-up.The discovery of hepatomegaly in a patient with poorly controlled type 1 diabetes mellitus must evoke a variety of diagnoses. Although hepatic glycogenosis is a rare diagnosis, it should not be ignored by the clinician.Through these clinical cases, the pathophysiology of hepatic glycogenosis, complications as well as therapeutic management has been discussed.