Spontaneous arteriovenous communications below the diaphragm is a very rare condition. Its association with polysplenia has perhaps not yet been reported in children. We reported a case in a 9-year-old boy presenting with acute onset of fever, vomiting, headache, seizures and altered sensorium. A CT scan revealed a large occipital abscess which was drained surgically. On examination he had marked central cyanosis and grade III clubbing of fingers and toes. Further investigations led to the discovery of a very rare finding of abdominal arteriovenous malformation associated with multiple-enhancing structures around the splenic bed suggestive of polysplenia. We believe that the most likely cause of the brain abscess was paradoxical embolism through the abdominal arteriovenous communication. Unfortunately the parents did not consent to a surgical correction of the malformation, realising the risks involved in the procedure. Subsequently the boy was lost in follow-up.