Patients with pelvic kidneys are at an increased risk of developing ureteropelvic junction obstruction (UPJO) and nephrolithiasis with limited endourologic options. A 54-year-old man with a left pelvic kidney, recurrent nephrolithiasis, and 12 previous ureteroscopies presented with left UPJO and lower pole calyceal stones. After two failed ureteroscopic attempts, an open pyelolithotomy and pyelocystostomy were performed. After 30 months, he continues to be asymptomatic without recurrence of nephrolithiasis. This constitutes the fifth such reported case. Therefore, pyelocystotomy is a good option for patients with pelvic kidneys, UPJO and recurrent nephrolithiasis refractory to endourologic procedures.