Diaphragmatic defects such as eventration and hernia are known to be associ
ated with a high-sited, sometimes intrathoracic spleen. We report here a un
ique case of an 8-year-old boy found to have a left congenital diaphragmati
c eventration and a suprapubic wandering spleen after presenting with sympt
oms of an "acute abdomen" due to torsion of the splenic pedicle. To our kno
wledge only one other case of a similar paradoxical association of these an
omalies has been reported before; interestingly, in this patient splenic in
farction had also resulted secondary to splenic torsion. We recommend caref
ul evaluation of the location and vascular status of the spleen in all pati
ents with congenital diaphragmatic defects due to the common occurrence of
splenic vascular insult.