Lynch syndrome is a rare pathology triggered by several genetic mutations that predispose individuals to various tumors. Although uncommon, urothelial carcinoma of the upper urinary tract can present with different characteristics. Here, we present the case of a patient with Lynch syndrome who developed a multifocal, recurrent lesion. A young woman with Lynch syndrome underwent screening at 30 years of age. She presented with macroscopic hematuria, underwent a urinary tract ultrasound, and was diagnosed with bladder cancer. The lesion was resected, and after a few years, she presented with a tumor of the right renal pelvis. Subsequently, a laparoscopic radical nephroureterectomy was performed. As the patient had an allergy to iodinated contrast, we continued screening with cystoscopy, urinary tract ultrasound, and oncotic cytology in the urine. After several years, she developed a new recurrence in the left renal pelvis and left ureter. Despite multiple tumors, as the patient had a single kidney, a therapy with lower morbidity was selected. This comprised endoscopic resection of the lesion and laser ablation at its base. After years of follow-up, the patient showed no evidence of disease recurrence and remained under follow-up. Lynch syndrome is a pathology with the potential for multisite tumors. It is extremely important for urologists to understand this syndrome because urothelial tumors of the upper urinary tract may be the initial manifestation of this syndrome. Screening must be individualized for each patient, considering the limitations of each method.