Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune-mediated disorder with heterogeneous multiorgan manifestations. Early identification and treatment of IgG4-RD are crucial for organ function recovery. Rarely, IgG4-RD manifests as a unilateral renal pelvic soft tissue mass that may be misdiagnosed as urothelial malignancy, resulting in invasive surgical intervention and organ damage. Here we present a 73-year-old man who had a right ureteropelvic mass with hydronephrosis detected by enhanced computed tomography. Right upper tract urothelial carcinoma and lymph node metastasis was highly suggested based on the image findings. However, IgG4-RD was suspected due to his past history of bilateral submandibular lymphadenopathy, nasolacrimal duct obstruction, as well as a high serum IgG4 level of 861 mg/dL. The ureteroscopy with tissue biopsy showed no evidence of urothelial malignancy. His lesions and symptoms improved after glucocorticoid treatment. Hence, a diagnosis of IgG4-RD was made, with the phenotype of classic Mikulicz syndrome with systemic involvement. The manifestation of IgG4-RD as a unilateral renal pelvic mass is rare and should be kept in mind. A ureteroscopic biopsy and serum IgG4 level measurement can help in the diagnosis of IgG4-RD in patients with a unilateral renal pelvic lesion.