Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare type of epidermal nevus characterized by an inflammatory appearance, intense pruritus, and a tendency to be resistant to treatment. We present here, the case of a 7-year-old boy who presented with a widespread inflammatory skin lesion on the right side of his body, with some of the eruption following Blaschko's lines. He also had an associated contracture in the ipsilateral limb and short stature. Initially, we suspected this may be a rare case of CHILD syndrome in a boy. However, after further investigation, we confirmed the diagnosis as a severe form of ILVEN.