Sporadic Meningioangiomatosis Presenting as a Middle Cranial Fossa Arachnoid Cyst

BACKGROUND: Meningioangiomatosis is an extremely rare meningovascular disease of the central nervous system that is characterized by the proliferation of leptomeninges, cortical vessels, and perivascular spindled cells. Although it is a benign, neoplastic disorder that carries a good prognosis after surgical excision, initial diagnosis may be challenging as radiologic findings are often variable and nonspecific. CASE DESCRIPTION: In this report, we describe an unusual presentation of meningioangiomatosis presenting as a symptomatic middle cranial fossa arachnoid cyst. CONCLUSIONS: In view of the unexpected diagnosis and infrequency of this condition, the case is discussed in collaboration with current literature and management strategies.