OBJECTIVE To investigate the potential role of tumor enucleation (TE) for the treatment of giant angiomyolipomas (AML). METHODS We retrospectively analyzed a prospectively derived database of 707 patients with kidney tumor, who were treated with conservative surgery, between January 1995 and September 2009. Overall, 31 patients had a histopathologic diagnosis of renal AML and of those, 3 patients had a diagnosis of unilateral or bilateral renal mass, with at least one clinically suggestive of giant AML (maximal tumor diameter > or = 9 cm), either central or perihilar. These patients were the subjects of the analysis. Nephron sparing surgery (NSS) was performed as tumor enucleation (TE), carried out by a blunt dissection, using the natural cleavage plane between the tumor and the normal parenchyma. RESULTS Preoperative tumor size ranged between 9.0 and 15.0 centimetres. At surgery, after kidney capsule incision, TE was done in all cases. In critical surgical steps, in case of difficult visualization of the correct enucleation plane, a sharp dissection a few millimetres away from the tumor was adopted. Warm ischemia time (WIT) was always below 20 minutes. Intraoperative blood loss was negligible. Unsignificant postoperative creatinine variation was recorded in all cases. No intra- and post-operative complications occurred. At last follow up visit, no tumor recurrence at the enucleation site was reported. CONCLUSIONS TE technique can be considered a viable and effective treatment option for this very rare pathologic condition, since it provides a maximal glomerular preservation and minimizes WIT and intra- and postoperative complications.