Six patients are reported with hypernephroma and various combinations of hepatomegaly, splenomegaly, and hepatic dysfunction without hepatic metastases or other factors to account for the abnormalities. Typical findings included fever, constitutional symptoms, weight loss, hepatosplenomegaly, anemia, occasionally thrombocytosis and monocytosis, and abnormal liver function tests characterized particularly by hypoalbuminemia, hyperglobulinemia, abnormal sulfobromophthalein (BSP) retention, and striking elevation of serum alkaline phosphatase activity. In some cases abnormalities regressed after removal of the tumor, and in others the diagnosis was made unexpectedly at autopsy, at which time the tumor was found not to involve the liver. A search of the literature disclosed 18 similar cases, bringing the total to 24 examples of the "syndrome." Therefore, it is suggested that hepatosplenomegaly and hepatic dysfunction may occur as manifestations of hypernephroma without metastases, and the abnormalities may regress after nephrectomy.