Cystic partially differentiated nephroblastoma (CPDN) is among the rare kidney tumors of childhood. Here, a case of CPDN in a baby who was operated with the diagnosis of multicystic kidney is reported. Clinical and imaging studies have a limited role in the differential diagnosis and diagnosis of this entity. A careful histopathological study is required to distinguish between CPDN and other types of multicystic kidneys. Because of the rare potential for aggressive behavior of this tumor, nephrectomy is the treatment of choice alone.

In this case presentation, we report a case of 13-year-old girl who was referred for treatment with vesicoureteral reflux (VUR), who had complaints of frequent urinary tract infections and urinary incontinence since her birth, and was diagnosed with congenital vesicovaginal fistula (CVVF). In the fistula closure procedure, multilayer repair and uretereneocystostomy with a transtrigonal approach was performed, and the patient did not have any complaints after the treatment. CVVF can usually be seen with other urogenital system anomalies. We aimed to present our unique case because of the challenging in diagnosis and association with VUR.

Venous tumor thrombus is a known manifestation of Wilms’ tumors in a minority of cases but has not been previously described in association with cystic nephroma. We report an original case of a histologically-benign cystic nephroma presenting with venous tumor thrombus extending to the inferior vena cava. The tumor thrombus was not detected by preoperative Doppler ultrasound. The patient was successfully treated with radical nephrectomy, tumor thrombectomy, and IVC closure. Postoperatively, the patient underwent genetic testing which revealed a DICER1 mutation—known to predispose affected individuals to a variety of benign and malignant tumors—and requires intensive surveillance for associated conditions.

Cystic lymphangioma is a benign, unencapsulated tumor caused by congenital malformations of the lymphatic system. It is a rare type of hematoma that unusually causes scrotal swelling. A 3-year-old boy presented with a painless swelling in his right hemiscrotum that gradually increased over a year. The swelling was clinically evaluated as a hydrocele, but a multi-localized cystic mass filled with hemorrhagic fluid was detected on surgical exploration. A complete excision was made and histopathological evaluation revealed cystic lymphangioma. As a result, cystic lymphangioma mimicking hydrocele can be seen in children. Although it is a rare type of tumor, this pathology should be suspected during the surgical operation in the groin area.

Ureteral anomalies are difficult to diagnose on routine investigations in the background of a megaureter. Ureteric valves are a rare cause of congenital obstructive uropathy. Mid ureteric valves are rarer than proximal or distal ureteric valves, and most patients are misdiagnosed preoperatively. Therefore, intraoperative identification is critical. We present a case of mid ureteric valve which presented as unilateral hydroureteronephrosis.