A case of umbilical endometriosis is presented to highlight the challe
nges in its diagnosis. The etiology, clinical findings, histologic eva
luation, prognosis and treatment options are discussed. While cyclic s
ymptomatology may lend evidence to the diagnosis of umbilical endometr
iosis, history and clinical findings are often equivocal. Decidualizat
ion of umbilical endometriosis can be initially confused with a malign
ant process on histologic evaluation. The potential for malignant dege
neration appears low. Surgical intervention is the treatment of choice
.