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IntroductionA wandering spleen occurs when there is a laxity of the ligaments that fix the spleen in its normal anatomical position.Case PresentationThis is a case report of a wandering spleen with horseshoe kidney in a 29-year-old male admitted with acute lower abdominal pain and vomiting to emergency department of Shariati hospital in Isfahan province. Sonographic examination showed a homogeneous 21 × 15 × 8 cm mass in the lower part of the abdomen and pelvis associated with a horseshoe kidney. Laparotomy confirmed the clinical and ultrasound findings.ConclusionsThe association of horseshoe kidney with a wandering spleen in this case may be due to an embryological anomaly.