An extremely rare case of a 39-year-old man with Nelson’s syndrome and paratesticular tumours is presented. The patient had undergone bilateral adrenalectomy at the age of 23 years for Cushing’s syndrome. Later he developed skin and mucosal hyperpigmentation, Nelson’s syndrome was diagnosed, and he underwent transfrontal hypophysectomy. At age 29, the presence of paratesticular tumours was noted which were excised together with the left testis. The preoperative levels of testosterone, produced by the paratesticular tumours, were extremely high; they fell after surgery.