A rare case of adrenogenital syndrome due to adrenocortical tumor is reported in a male child with left-sided hemihypertrophy and the subsequent development of an embryoma.

Marked regression of hirsutism, advanced somatic and muscular development, deep voice and elevated urinary 17-ketosteroids followed removal of an adrenocortical adenoma. An embryoma was discovered five years later near the site of original operation. Removal of this tumor was followed by metastases and death after 15 months.

The unusual association of adrenogenital syndrome with hemihypertrophy and embryoma is discussed. This case serves to emphasize the need for a guarded prognosis and alert observation even years after an uneventful course following removal of an adrenocortical tumor.

A brief review is presented of 22 cases of adrenogenital syndrome in male children with adrenocortical tumors including the above case.

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