Spontaneous perirenal heamatoma is a rare medical emerency and is difficult to diagnose early. It mostly occurs secondary to rupture of a renal tumor (61.5%) and vascular disease (17%). We report a case of 28 year-old man without a history of systemic disease or trauma, who had progressive severe right flank pain without hematuria or hydronephrosis fir one day. He was initially diagnosed as having muscle pain, and then a hepatic tumor with intra-tumor bleeding was suspected on follow-up abdominal sonography. Finally, a right renal angiomyolipoma rupture with perirenal heamatoma was proved pathologically alter surgical exploration. The patient was discharged without complications after enucleation of the angiomyolipoma in the right kidney.