Aim: Macrodystrophia lipomatosa is a rare cause of gigantism of limb which can be confused with other common causes like congenital lymphedema. It presents usually with loss of function and cosmetic problems. Four cases are described with emphasis on clinical presentation, differential diagnoses, imaging and treatment options.
Methods & results: Four patients of macrodystrophia lipomatosa were thoroughly examined and subjected to investigations.
Conclusion: Besides diligent clinical examination, imaging and histopathology are crucial in clinching the diagnosis.