Macrodystrophia lipomatosa (MDL) is a rare congenital variant of focused gigantism that is non-hereditary. Typically, MDL presents with localized gigantism in either the hand or foot. In this case report, we present the unique instance of a 12-year-old girl who has experienced enlargement of the first and second toes on her right foot since birth. Plain radiographs and MRI findings revealed the accumulation of fatty tissue around the first and second toes, medial and lateral aspects of the first metatarsal, extending up to the medial plantar arch of the foot. To enhance foot functionality and alleviate any issues with wearing footwear, a successful reconstruction surgical intervention was performed. As a result, the patient can now wear shoes without any difficulties. MDL is a very uncommon kind of congenital localised gigantism, and surgical consultation is frequently performed for cosmetic reasons.