Madelung’s disease is a rare metabolic disease of surgical importance seldom reported among Africans. This case reports the condition in a middle-aged Nigerian man with Type 1 variety of the condition. In addition, he was overweight and had diastolic hypertension (90 mmHg) with a 30-year history of local alcohol consumption of 30-60 units/day. Diagnosis of Madelung’s disease was made by fine-needle aspiration cytology, ultrasound scan, and x-ray of the fatty deposits on the body. The patient also had an aspartate transaminase-to-alanine transaminase (AST/ALT) ratio of 2.0, indicative of alcoholic fatty liver disease. Management was through counseling to stop alcohol and excision of the neck mass. The case introduces this rare condition, demonstrates the need for evaluation of patients with Madelung’s disease for metabolic complications, and highlights the role of interdisciplinary collaboration in the management of this disease.