Background: The association of multiple cutaneous granular tumors with systemic defects is extremely rare. To date, 14 cases have been described. Case report: A 14-year-old boy presented multiple nodular lesions on his arms and legs; facial dysmorphism and joint hypermobility were also present. Histopathologic examination of two nodules showed granular cell tumors. We review the literature regarding multiple granular cell cutaneous tumors in children with associated somatic defects and genetic syndromes. Conclusions: The combination of multiple cutaneous granular tumors with alterations in other organs is not only fortuitous, justifying the proposal of a distinct syndrome, termed “ Bakos’s syndrome” after the author who first described this association. The relation with neurofibromatosis type 1 is discussed, and an association is suggested with the range of variants of this syndrome. Background: The association of multiple cutaneous granular tumors with systemic defects is extremely rare. To date, 14 cases have been described. Case report: A 14-year-old boy presented multiple nodular lesions on his arms and legs; facial dysmorphism and joint hypermobility Histopathologic examination of two nodules showed granular cell tumors. We review the literature regarding multiple granular cell cutaneous tumors in children with associated somatic defects and genetic syndromes. Conclusions: The combination of multiple cutaneous granular tumors with alterations in other organs is not only fortuitous, justifying the proposal of a distinct syndrome, termed “Bakos’s syndrome” after the author who first described this association. The relation with neurofibromatosis type 1 is discussed, and an association is suggested with the range of variants of this syndrome.