An aggressive destruction pattern resulting in joint deformation has not been described in skeletal cystic angiomatosis (SCA) so far. We present the case of a 6-year-old boy with such findings strongly resembling Gorham's disease (massive osteolysis). Since the prognosis of the latter entity tends to be less favorable than in SCA, particularly SCA without extraskeletal involvement, careful differentiation of both disorders appears to be important.