The accessory spleen is a congenital disability characterized by a separated ectopic splenic parenchyma. Wandering spleen (WS) is a rare condition where the spleen, free from its ligaments, is allowed to move inside the abdomen, predisposing the patient to life-threatening complications due to torsion of the vascular pedicle; splenic infarction, portal hypertension, bleeding and acute abdomen may occur. WS or Accessory Spleen (AS) is rarely suspected at presentation since symptoms are usually not specific, and definitive diagnosis is often reached only by imaging technologies such as color flow ultrasonography and angio-spiral computed tomography. The size is rarely more than 4cm. Patients rarely present symptoms, and the diagnosis can be accidental when faced with ectopia in radiological or intraoperative investigations. It is an essential clinical entity since abdomen-pelvic tumors must always be considered as differential diagnoses, requiring detailed evaluation. Surgical treatment is indicated for symptomatic patients or those with complications. On the other hand, the ectopic kidney with rotation abnormality also consists of a singular entity. Congenital anomalies of the upper urinary tract occur in 3%-4% of those born, most of which are followed up clinically. The present study discusses a case of symptomatic intrapelvic WS associated with an ectopic kidney with rotation anomaly, a rare union, and still with scarce reports in the literature. This study aimed to present the case of a patient with an isolated oversized accessory spleen and a review of the literature.

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