Abstract Background The coexistence of Rheumatic Heart Disease (RHD) and Pulmonary Arteriovenous Malformation (PAVM) is a rare clinical scenario that poses diagnostic and therapeutic challenges. This case report explores the clinical presentation, diagnostic journey, and multidisciplinary management of a patient presenting with both conditions. Case Summary A 47-year-old female with a history of RHD presented with symptoms of dyspnoea on exertion and cyanosis, suggestive of both cardiac and pulmonary involvement. Subsequent investigations involving imaging, echocardiography, and invasive pulmonary angiography, revealed the co-existence of RHD and multiple PAVM in patient’s left lower lobe of lung. The patient underwent a tailored treatment plan, initially involving percutaneous mitral balloon valvuloplasty for RHD, followed by a staged procedure of transcatheter PAVM closure with Amplatzer™ Vascular Plug II performed one month later. Her saturation normalized following intervention. The patient's progress was monitored closely, with adjustments made to the treatment plan based on evolving clinical scenarios. Patient remained well in short term follow up. Discussion This case highlights the complexity of managing patients having two diverse conditions RHD and PAVM coexisting together, thus emphasizing the importance of a multidisciplinary approach. The unique intersection of cardiac and pulmonary pathologies necessitates careful consideration of diagnostic nuances and tailored treatment strategies. Lessons learned from this case offer valuable insights for clinicians encountering similar scenarios and underscore the significance of individualized, patient-centred care in optimizing outcomes for those with dual pathologies.