Introduction: The case is reported of an extremely rare primary splenic lymphoma (PSL) and summarizes the basic concepts and techniques of contrast enhanced ultrasonography (CEUS), focusing on splenic applications for the diagnosis of malignant splenic lesions. Case report: A 52-year-old male with no co-morbidities presented with a history of left upper abdominal pain for one month, and chronic fatigue. Abdominal ultrasound was performed in which suspicious focal lesions in the enlarged spleen was detected, and assessed with contrast enhanced ultrasound, followed by magnetic resonance imaging (MRI). The diagnosis of malignant splenic tumour was made based on history, clinical examinations and radiographic appearance in CEUS and MRI. Conclusions: In the presented case contrast enhanced ultrasound allowed establishing the diagnosis of malignancy within the spleen, which was later confirmed with MRI and histopathological examination