Langerhans-cell histiocytosis (LCH) is a condition characterized of an inadequate function and proliferation of this cells. Clinical presentation can be variable depending on the site of affection. The most common sites of affection are bones, skin and lymph nodes; however, this histiocytic disorder can arise in virtually any organ system, making it challenging to diagnose. In this case report we describe a 6-month-old with an atypical and unique presentation of multisystemic LCH: skin involvement and pericardial effusion. We highlight the importance of clinical suspicion among physicians when symptoms of several systems appear, especially when skeletal or skin systems are affected.