Some neoplastic polyendocrine syndromes may affect the kidney. These include multiple endocrine neoplasia type 1 and 2 (MEN 1, MEN 2) as well as mixed syndromes characterized by endocrine and non-endocrine diseases. Kidney involvement may be related to secondary systemic hypertension or diabetes mellitus, direct hormonal effects, and benign or malignant kidney tumors or kidney malformations (very rare). Neoplastic polyendocrine syndromes are rare and it is important that the endocrinologist knows the possible renal complications or associated diseases for correct screening, and that the nephrologist is aware of the need for endocrinological assessment in young hypertensive patients who are resistant to therapy, hypertensive patients with adrenal lesions, and patients with hypercalciuria or renal calculi, especially at a young age.