Morphea (or Localized scleroderma) is an idiopathic disorder characterized by local inflammation followed by fibrotic changes in the skin and subcutaneous tissues. Despite a shared histologic appearance between systemic sclerosis and localized disease, the clinical distribution of lesions serves to distinguish these two disorders. Some literature recently reported systemic manifestations with morphea which resulted in changing concept for the nomenclature – using the term morphea in localized scleroderma and preference of the terminology. We present a 20 years old male patient with circumscribed markedly thickened skin lesion over neck, chest and part of the abdomen associated with systemic manifestations after investigations and after excluding other differential diagnosis. We propose using the term “morphea with systemic manifestations.