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A 91/2 year old female born of a nonconsanguineous marriage presented with macrodactyly, hypertrophy of the limb, lymphangioma circumscriptum and varicosities. Lymphangioma circumscriptum was confirmed by histopathology. X-ray of the right foot revealed hypertrophy of both subcutaneous tissue and the underlying bone. This is the second case report from India with the characteristic macrodactyly.