Gorlin's syndrome presenting with myolipoma of tongue base.

Objective: We report the first case of tongue base myolipoma associated with Gorlin's syndrome.

Method: Case report and review of world literature.

Results: A 39-year-old man with known Gorlin's syndrome presented with progressive dysphagia. Subsequent magnetic resonance imaging scan and biopsy confirmed the rare diagnosis of myolipoma arising from the tongue base. In view of the benign nature of this mass, it was debulked rather than completely excised in order to preserve swallowing function.

Conclusion: To our knowledge, this is the first report of the coexistence of two rare conditions in an atypical fashion. This case appears to represent a new variant in the broad spectrum of features of Gorlin's syndrome.