Multiple granular cell tumors associated with giant speckled lentiginous nevus and nevus flammeus in a child. We describe an 11-year-old girl in whom multiple cutaneous granular cell tumors were associated with a giant speckled lentiginous nevus and an extensive nevus flammeus. An association between granular cell tumors and pigmented skin lesions has been reported twice previously and supports a neural origin for these tumors. An abnormality of neural crest development is proposed to explain the coexistence of three uncommon and unusually extensive cutaneous disorders in this patient. This case may represent a further variant of phakomatosis pigmentovascularis. 