Introduction:Edheim Chester Disease (ECD) is a rare disease of the non-Langerhans histiocytosis category involving organs such as the long bones, Central nervous system (CNS), skin, kidney, heart, arteries, and endocrinopathies.(1, 2) The disease is usually more common in the 50-60 years and men. Mutations that disrupt the cellular RAS-RAF-MEK-ERK signaling pathway play an essential role in the pathology of this disease.(3) Also, those diagnosed with the BRAF VR600E- mutation have more cardiac and CNS involvement.(4) Cardiac involvement in these patients can be seen as pseudo-tumor infiltrates of the right atrium (RA) in 36% of patients.(3)Diagnosis of the disease is based on histo-pathological, medical, and radiological findings, among which MRI is a key part of diagnosing the condition. (7, 8)In this clinical case, we present a challenging scenario involving a 42-year-old male patient who presented with shortness of breath and skeletal pain, in which a multi-disciplinary approach plan led to the diagnosis of ECD..