We report clinical, radiological, and pathological features of three cases of macrodystrophia lipomatosa, which is characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. The peculiarity in our report is the involvement of contralateral hand and feet in our 1(st) case in contrast to usual presentations of this rare condition, which is often unilateral. In our 2(nd) case, the lateral aspect of foot was involved as against the usual finding of involvement of medial aspect in lower limbs, also another surprising finding in this case is increase in the size of lesion after puberty. Coming to our 3(rd) case, enlargement of almost whole of an upper extremity with profound involvement of middle, ring and little finger along with total sparing of the thumb is in itself an extremely rare occurrence as in upper limb, mainly the lateral aspect of hand and related digits bear the burnt and more over involvement of whole limb is itself an unique phenomenon.
Keywords: Fibroadipose; localized gigantism; macrodystrophia lipomatosa.