Giant hydronephrosis (GH), characterized by the presence of more than 1 L of fluid in the renal collecting system, is a rare urological condition, particularly in adults. Obstruction of the pyeloureteral junction is the most common cause of GH. We report the case of a 51-year-old man who presented with dyspnea, edema of the lower limbs, and major abdominal distension. The patient was diagnosed with obstruction of the pyeloureteral junction, causing a left giant hydronephrotic kidney. After renal drainage of 27 L of urine, a laparoscopic nephrectomy was performed. GH usually manifests as asymptomatic abdominal distension or vague symptoms. However, very few published reports describe cases of GH initially presenting with respiratory and vascular manifestations.