Macrodystrophia lipomatosa is a rare, congenital, non-hereditary disease, characterized by local gigantism of the fingers or toes. We report the case of a 37-month-old boy, with no prior past medical history, who presented with a gigantic dystrophy of the left forefoot. The location of the deformity was involving the plantar and dorsal aspect of the foot, and digits 1 to 4. After clinical examination, imaging study assessment, and differential diagnosis considerations, it was established that macrodystrophia lipomatosa was the cause of the deformity. A reconstructive surgical intervention was planned. The hypertrophied tissues were excised, resulting in a reduction in the forefoot's volume. The patient had a favorable postoperative course and ambulation was allowed with a custom-made shoe. The case represents a rare pathological entity with complex diagnostic and therapeutic considerations. As far as treatment options, the surgeon must decide between a reconstructive surgical intervention and amputation.