A 39-year-old woman with Carney complex presented with a stroke of undetermined etiology. Computed tomography showed bilateral thalamic infarctions and also an unsuspected multicompartmental cystic neoplasm that had eroded the anterior clivus and extended forward into the nasopharynx. Histologically, the mass appeared benign and was composed of spindle cells and multiple foci of striated muscle. Immunohistochemically, the spindle cells were strongly reactive for S-100 protein and to a lesser extent for CD57, collagen IV, neuron-specific enolase, smooth muscle actin, epithelial membrane antigen, and glut-1. The striated muscle cells were positive for desmin and myogenin. The MIB-1 labeling index was 0.5%. Ultrastructural examination was necessary to reveal the full extent of divergent differentiation. Ultrastructurally, the spindle cells showed divergent differentiation along several cell lines, including smooth muscle, neuroendocrine, hybrid smooth muscle-neuroendocrine, perineural-like cells, and striated muscle. The occurrence of this unique lesion in a patient with the Carney complex raises the possibility that it may be a rare component of the syndrome.