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Varying sizes of renal angiomyolipoma (AML) have been reported in the literature, with the general consensus that a size greater than 10 cm can be termed a giant AML. Our patient presented with increasing abdomen girth since last 3 years. Contrast-enhanced computed tomography scan of the abdomen confirmed the presence of “AML” arising from the upper and mid pole of the right kidney with similar small foci in the contralateral kidney. The patient underwent nephron-sparing surgery with preserving the right lower pole with enucleation of multiple small foci in the right kidney. The surgical specimen, which was confirmed to be AML histopathologically, was 40 cm × 20 cm × 15 cm in size and 7000 g in weight. This represents the case of the largest AML with a dimension of up to 40 cm and managed by nephron-sparing surgery without prior angioembolization. AML larger than 10 cm is usually considered to be giant AML in the literature.