Sclerosing angiomatoid nodular transformation (SANT) of the spleen is an uncommon primary benign condition with specific histopathological characteristics. The majority of the patients are usually asymptomatic and present for treatment after an incidental finding on a routine abdominal imaging. The differential diagnosis includes other benign tumours, as well as primary malignancy or metastases of unknown origin. We report a 55-year-old woman who was presented to the surgical team of our hospital for splenectomy after the investigation of an incidental splenic lesion led to the diagnosis of SANT. The pathology report of the specimen repeated the initial diagnosis. The patient has not yet presented any complication or recurrence of the disease 8 months after the operation. From such infrequent cases, the most valuable conclusion which can be yielded is that the more the number of patients reported, the more the knowledge about the disease is broadened.