Abstract A boy, 9 years, 8 months of age, with hairy pigmented nevi over the trunk, upper
extremities, and face with bone deformities characteristic of rickets was admitted because of
general weakness and poor appetite for 3 weeks. Repeated examinations demonstrated
marked brainstem dysfunction; a brainstem tumor was visualized by computed tomography.
The patient died 14 days after admission despite supportive treatment. The relationship
between giant intradermal nevocellular nevi and brain tumor is discussed.