We report two extremely rare cases of isolated sphenoid 'fungal ball' in conjunction with vision loss. Case reports and a literature review concerning diagnosis and management of isolated sphenoid fungal ball in conjunction with vision loss. A mycetoma or fungal ball resulting from isolated sphenoid fungal sinusitis is a rare disease. It is frequently difficult to diagnose, as patients present with nonspecific symptoms such as headache, visual disturbance and cranial nerve palsies. Diagnosis of the disease is typically not made until advanced imaging has been performed. We present two cases of patients with sphenoid sinus fungal ball who developed headaches and vision loss. One patient had symptoms of IIIrd cranial nerve palsy and the other partial visual field impairment. After a timely sphenoidotomy, both patients recovered fully, with resolution of all symptoms. These two cases of sphenoid fungal ball in conjunction with vision loss suggest that the otolaryngologist should keep this disease in mind even when symptoms are nonspecific. Endoscopic surgery can be a safe and viable therapeutic approach for this condition.