We report the case of a 4-year-old boy who presented to our facility with an enlarged right second toe. After radiography ruled out acute pathology, an MRI showed increased adipose tissue at the distal aspect of the appendage. Although the parent felt that the appendage was unaesthetic, there was no functional issue, and further discussion with the pediatricians and pediatric surgeons eliminated the need for immediate intervention. Macrodystrophia lipomatosa is a rare entity that predominantly affects children. Distributed evenly in males and females, the disease refers to hypertrophy of fibroadipose tissue of the distal upper and lower extremities.