A 33-year-old woman presented with nonspecific, colicky pain of the left lower abdomen. Computed tomography (CT) revealed a 2-cm mass engulfing the mid-left ureter. Ureteroscopy and biopsy revealed normal mucosa, and CT-guided biopsy of the mass was nondiagnostic. The patient underwent laparoscopic exploration. A frozen section taken from the mass revealed a granular cell tumour. We excised the whole involved portion of the ureter and performed end-to-end ureteroureteral anastamosis. The postoperative course was uneventful. Examination of a segment of resected ureter revealed a granular cell tumour diffusely infiltrating the wall of the ureter. There were no features suggesting a malignant phenotype. On follow-up, the patient was found to have developed a stricture at the anastomotic area, which was successfully treated with balloon dilatation. To our knowledge, this is the first reported case of a granular cell tumour involving the ureter.