Purpose: Because angiomyolipoma is less common in children than in adu
lts, its diagnosis can be difficult. We present 3 cases of pediatric a
ngiomyolipoma in which diagnostic problems resulted due to the present
ing characteristics. Materials and Methods: We report on 3 children wi
th unilateral renal angiomyolipoma. Computerized tomography (CT) and u
ltrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diame
ter, respectively. A correct diagnosis was not made preoperatively in
any case by CT, ultrasound or fine needle biopsy. Wilms tumor was susp
ected in the first patient who received preoperative chemotherapy. Ima
ging was inconclusive in the other 2 cases. Results: All patients unde
rwent surgical exploration and subsequent nephrectomy due to the large
size of the tumor. At followup 33, 23 and 13 months postoperatively a
ll children were well without signs of recurrence. Conclusions: It has
been reported that the demonstration of fat on renal ultrasound and C
T can diagnose angiomyolipoma in 95% of the cases. Most radiologists r
ely solely on CT demonstration of lipid density in the renal mass to d
iagnose angiomyolipoma but the identification at imaging of lipid tiss
ue may be difficult in small tumors. In our cases the fat content of t
he tumors was less than 10% despite the large size. This low fat conte
nt results in misdiagnosis, since fatty tissue is also present in othe
r renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors
. We recommend conservative surgery when tumor size permits in pediatr
ic patients with angiomyolipoma to avoid chemotherapy.