Macrodystrophia Lipomatosa (MDL) of the toe is a rare, congenital, disproportionate overgrowth involving one or more digits in the lower limb. Despite being a benign condition, when left untreated, it may cause physical impairment and interfere with daily activities. This form of localised gigantism is the result of excessive proliferation of fibroadipose tissue within the nerve along with associated macrodactyly. The mainstay of treatment is debulking or amputation to accommodate the patient's daily activities, as well as for cosmesis. In this case report, the clinical and radiographic findings, anatomical descriptions, and histopathological findings are presented. The difference between MDL, fibrolipomatous hamartoma (FLH) and lipomatosis of the nerve (LON) are also discussed.