Stewart–Bluefarb syndrome (SBS) is a subtype of pseudo-Kaposi sarcoma (PKS), which is associated with congenital arteriovenous malformation. It is a rare syndrome, characterized by cutaneous lesions with onset in the second decade of life. We report a case of SBS in a 33-year-old man presenting with a 15-year history of unilateral, reddish-brown, raised lesions on his right lower leg and the dorsum of his foot. Diagnosis of PKS was confirmed by histopathology and radiology. We report the clinical features, aetiopathogenesis, differential diagnosis and treatment of this rare condition.