Splenogonadal fusion (SGF) is a rare congenital malformation character
ized by fusion of the spleen and a gonad (almost always the left one)
frequently associated with orofacial and/or limb developmental abnorma
lities, Only 125 cases were reported between 1883 and 1994. This repor
t concerns a case of SGF in a 20-yr-old woman with an accidental findi
ng of a splenic space-occupying lesion protruding into the lower abdom
en in ultrasound and CT, Radiocolloid spleen scintigraphy and SPECT pr
oved to be the best procedure to establish the correct diagnosis of SG
F, As SGF is often asymptomatic, more liberal use of splenic scintigra
phy is suggested in patients with congenital limb and/or orofacial ano
malies. SGF should be included among the differential diagnoses of lef
t abdominal, pelvic or scrotal masses.