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Splenogonadal fusion (SGF) is a rare benign malformation in which spleen is aberrantly attached to the gonads or mesonephric derivatives. This entity often presents with scrotal mass, inguinal hernia, or cryptorchidism. Herein, we report our experience with a boy who presented with a scrotal enlargement which later turned out to be SGF. Moreover, an accessory spleen was unexpectedly found in the left inguinal canal. To the best of our knowledge, this is the first report of SGF and accessory spleen in the literature. Although rare, SGF should be included in the complete differential diagnosis list of scrotal masses, especially in children. The use of frozen section pathology if available would aid the surgeon in avoiding unnecessary orchiectomy in small testicular masses of benign origin.

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