Purpose <p>We are reporting on a rare case of a primary manifestation of Alveolar Echinococcosis in the spleen in a German patient.</p> Methods <p>The patient was initially presented to her gastroenterologist and subsequently referred to a district hospital for surgical management. The further antiparasitic treatment was taken over by the Institute of Infectious Diseases and Tropical Medicine, LMU University Hospital Munich, Germany.</p> Case report <p>A 59-year-old German woman presented to her gastroenterologist with weight loss and abdominal discomfort. An ultrasonographic finding of a splenic lesion was further evaluated by abdominal CT and MRI, interpreted initially as a hamartoma by the radiologists. Following partial splenectomy, where an accidental intra-operative cyst rupture occured, pathological examination raised suspicion of echinococcosis. An immune-histological analysis of the resected splenic tissue by the German Reference Laboratory for Echinococcosis confirmed Alveolar Echinococcosis. No additional lesions suggestive of echinococcosis were identified. The patient is currently successfully treated with continuous antiparasitic therapy with albendazole.</p> Conclusions <p>Primary manifestations of Alveolar Echinococcosis in the spleen are rare, and may be misleading towards other differential diagnoses.</p>