This is a unique case of a young patient who presented with signs and symptoms of Cushing's disease and was diagnosed post‐surgery as “corticotrophin tumor/adenoma associated with primary intracranial sarcoma, DICER1‐mutant.” Primary intracranial sarcoma, DICER1‐mutant (PIS‐DICER1) is a rare molecularly defined entity. Here, DICER1 is linked to adenoma.