Madelung's disease (MD) is a rare lipid metabolic disorder of adipose tissue overgrowth, which has been reported to be related to alcohol abuse. Although it does not affect survival itself, alcoholism and metabolic disorders associated with MD can be life-threatening. Although surgical procedures and classifications have been reported, long-term follow-up has rarely been reported. Here, we report a 61-year-old Japanese man with MD who has been followed-up for 12 years. Lipectomy was performed three times over the first 3 years and a total of 4 kg of adipose tissue was excised. Relapse has not been observed. He was diagnosed with multiple hepatocellular carcinomas (MHC) and placed on dialysis due to hepatorenal syndrome caused by alcoholism. Moreover, one of the MHC ruptured resulting in hemorrhagic shock. The cause of death in MD is not fat proliferation but comorbidities. Long-term observation and multidisciplinary systemic management are necessary for MD patients.