Sarcoidosis is a multisystem granulomatous disease with polymorphic manifestations, of unknown etiology, that can affect any organ in the body, but most commonly the lungs [1,2,3]. Extrapulmonary involvement is common, can occur in association with or in the absence of intrathoracic disease, and all organs can be involved, but it is rare to find an isolated extrapulmonary disease (less than 10% of patients) [3,4]. On occasion, the presentation of sarcoidosis may be atypical. We report a case of systemic sarcoidosis, with particular clinical data: extrathoracic onset with subdiaphragmatic lymph nodes, hepatomegaly and splenomegaly. Personal history and family history were insignificant.The onset was insidious, with two months prior this admission, with progressive weakness to which weight loss (10 kg) and nonspecific upper abdominal pain were associated later.His physical examination revealed only hepatomegaly and splenomegaly.Laboratory studies indicated biological inflammatory syndrome (ESR, slightly elevated fibrinogen), hypergammaglobulinemia and a negative tuberculin purified protein derivative (PPD ) skin test.Abdominal ultrasound and CT scan documented multiple subdiaphragmatic lymph nodes (periaortic, inter-aorto-caval and iliac), with diameter from 1.75 to 2.5 cm (Figure 1).