BACKGROUND Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign disease of the splenic red pulp of unknown etiology. Definite diagnosis is made on histopathology alone as it has no diagnostic radiologic characteristics. CASE REPORT We present a case of a large incidental splenic mass in a middle-aged man, whose refractory anemia resolved with splenectomy. Our initial imaging studies failed to differentiate this lesion from other splenic lesions like hamartoma and haemangioma. To the best of our knowledge, no SANT cases in the literature have been investigated with red cell scan, as performed in our patient, nor has any case had resolved anemia after treatment. Splenectomy was offered as malignancy could not be safely excluded. Histopathology confirmed the diagnosis of SANT. The patient made good recovery and had no signs of recurrence 2 years later. CONCLUSIONS SANT of the spleen is a rare condition that can mimic many other splenic tumors and poses diagnostic challenge when histopathology is unavailable. Our case adds to the number of SANT reported in literature in the hope of elucidating the pathophysiology of this rare condition.