Neonatal lupus (NL) is an acquired autoimmune disease of the newborn, caused by transplacental passage of the maternal autoantibodies anti SS-A/Ro and anti SS-B/La. When the clinical picture starts directly at birth, it is known as congenital lupus (CL). The clinical manifestations are variable. Except for cardiac involvement, the other manifestations tend to be benign, and resolve with the child's clearance of maternal antibodies. We report a patient who presented at birth with very severe involvement of the skin, and subsequent contractures of hands and feet, leading to functional impairment. The patient underwent surgical procedures with excellent result. At the age of 18 months, the patient was diagnosed with oligoarticular juvenile idiopathic arthritis (JIA), and bilateral uveitis at the age of 3 years and a half. Our aim is to alert health professionals about the possibility of a severe course of cutaneous manifestations in CL, as well as the role of CL and NL regarding development of other autoimmune diseases.