Congenital localized gigantism in children is rare and may be caused by a gamut of disorders. Family history, cutaneous and systemic manifestations and imaging together with histopathology assist in reaching the diagnosis. Macrodystrophia lipomatosa is one such rare non-hereditary congenital developmental anomaly causing localized overgrowth of a digit(s) or limb. Herein we report an interesting case of macrodystrophia lipomatosa in a child.