We present an exceptional association of splenogonadal fusion, Moebius syndrome, and intestinal intussusception. At the age of 1 year, the patient presented with vomiting, bloody stools, and abdominal distension. He underwent a laparotomy that revealed an ileo-ileal intussusception. Three days later, he underwent a new surgery for the reduction of a suspected inguinal hernia. A dark-red tubular structure consisting of splenic tissue was seen passing down through the processus vaginalis and attaching onto the left testicle. Owing to the rarity of the splenogonadal fusion, each case should be reported for a better knowledge of its etiopathogenesis, clinical characteristic and associations.