This  is  a  case  of  a  47  year-old  female  presenting  with  typical Cushingoid appearance and CSF rhinorrhea. MRI revealed a 4.4 cm x 2.9 cm x 4.5 cm enhancing intranasal mass with evidence of erosion of the left cribriform extending to the left anterior cranial fossa. Dexamethasone suppression  test  yielded  elevated  cortisol  level. Endoscopic nasal biopsy done showed a round cell tumor positive for  ACTH,  synaptophysin,  chromogranin  A,  and  S-100.  Patient subsequently  underwent  endoscopic  endonasal  excision  of  left intranasal mass with creation of pericranial flap for repair of CSF leak.  This  report  is  presented  to  discuss  a  rare  case  of  ACTH secreting esthesioneurblastoma including its diagnostic challenges and surgical options for repair of anterior cranial fossa defect to address CSF leak particularly by means of a vascularized pericranial flap.

Human ; Female ; Middle Aged ; Cerebrospinal Fluid Rhinorrhea ; Cranial Fossa, Anterior ; Synaptophysin ; Chromogranin A ; Hydrocortisone ; Nose ; Endoscopy ; Surgical Flaps ; Dexamethasone ; Adrenocorticotropic Hormone