Onset of features usually begins in childhood. Babies are typically born with a larger head, longer body, and usually weigh more than 9 pounds (4kg). After birth their growth slows and as a result, children and adults are of normal height and size. Sometimes children will also have decreased muscle tone and/or learning difficulties and developmental delay. Benign fatty tumors under the skin or in the abdomen and/or intestinal polyps (most commonly hamartomatous polyps on the intestine) are common in affected individuals. Hemangiomas, or raised red birthmarks caused by blood vessel changes, may also be present. Hemangiomas can be either internal (on the internal organs) or external (on the skin).

Patients diagnosed with Bannayan-Riley-Ruvalcaba syndrome do not necessarily have all the same features. Only 50 percent of children have developmental delays and learning problems, while the other 50 percent have normal development. Only 45 percent of affected individuals will develop polyps on the intestine. Page 3 lists the features that have been associated with Bannayan-Riley-Ruvalcaba syndrome and the percentage of people that develop each feature (incidence).

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