Introduction: Neonatal lupus is a syndrome diagnosed in newborns whose mothers suffer from systemic connective tissue diseases with the presence of antibodies to Ro(SS-A) and La(SS-B) antigens. It most often manifests with skin lesions and cardiac conduction disturbances. The aim of the study was to analyse the risk factors for neonatal lupus based on a literature review. In addition, a number of recommendations were formulated for prevention and further management of affected mothers and their children. Materials and methods: We reviewed PubMed, ResearchGate and Google Scholar databases. A combination of keywords: lupus, pregnant, infant, heart block (according to Medical Subject Headings, MeSH) was used to identify relevant publications. Results: Given the lack of protocols for the diagnosis and treatment of neonatal lupus, ensuring that women can monitor foetal heart rate and quickly access obstetric care in the event of abnormal readings is an important aspect in the early diagnosis of the disease. The question of prevention and treatment remains open, but given the results of recent studies, recommendations on prevention and treatment of this syndrome have been designed. Conclusions: 1. Prophylactic maternal intake of hydroxychloroquine. 2. Weekly doppler ultrasound of the umbilical artery and echocardiography. 3. Planning delivery in specialised health care units. 4. Follow-up of the infant until 6–7 months of age.