Sinus histiocytosis with massive lymphadenopathy （SHML） is a rare histiocytic proliferative disorder and mainly characterized by sinus histiocytosis with lymph node enlargement. Its clinical manifestations are related to the location and size of the tumor， and often include headache， dizziness， vomiting， vision loss， unsteadiness in walking， and decreased pituitary function in SHML involving the saddle area. The imaging manifestation is mostly a substantial swelling involving the dura mater with no specificity， and the magnetic resonance imaging （MRI） often shows iso-signal T1WI， low-signal T2WI， and obvious enhancement on enhancement scan. The MRI presentation in this case was consistent with that in conventional SHML， with the presence of reduced thyroid function， insufficient cortisol secretion， extensive lesions involving the meninges and surrounding tissues and involving the entire venous sinus， saddle area， and part of the skull， and both nodular and striated dural enhancement. After surgery， hormonotherapy， and immunotherapy， the patient’s dyspnea， headache， dizziness， unsteadiness in walking， and imaging manifestations were improved at 2-month follow-up compared with those before treatment.