Maxillary sinus melanoma as the presenting feature of Carney complex

We present a case of a large maxillary sinus tumor in a 6-year-old boy, immunohistologically indistinguishable from a malignant melanoma, that led to the diagnosis of Carney complex. The Carney complex is an autosomal dominant disorder characterized by mucocutaneous pigmented lesions and neoplasia of multiple endocrine glands and is usually due to an inactivating mutation of the gene for the protein kinase A regulatory subunit 1A. The Carney complex has characteristic head and neck manifestations that can point to the diagnosis of this potentially lethal condition.