Abstract: Macrodystrophia lipomatosa (MDL) is defined as rare congenital entity characterized by proliferation of mesenchymal elements predominantly the fatty component resulting in localized or regional enlargement of part or whole limb. It usually presents with cosmetic problems and variable functional deformity. Even though history and physical examination are usually diagnostic, radiological evaluation especially the cross-sectional modalities can reliably distinguish MDL from other forms of localized congenital gigantism.
Case Series: Here we are presenting a series of three cases of macrodystrophia lipomatosa (MDL) presented to radiology department with history of disproportionate focal overgrowth of limb.
Conclusion: Macrodystrophia lipomatosa is a rare form of congenital localized gigantism. As there are numerous etiologies of focal gigantism and clinical distinction may be difficult at times, radiological evaluation is very useful in confining the differential diagnosis. Imaging, with magnetic resonance imaging (MRI) in particular, provides vital clues to diagnose the condition which is confirmed by histopathology. Though surgical consultation is often offered for cosmetic reasons, it should be delayed till puberty or when functional problems exist as surgical results are not very rewarding with significant recurrence rate.

Keywords: Fatty overgrowth, Macrodactyly, Macrodystrophia lipomatosa, Soft tissue overgrowth