Cutaneous syndromes associated to malformations or pathologies of other organs are relatively common, but diverse with regard to their manifestations. In cases with uncommon features the diagnostic process may be difficult, and the histopathological analysis could be the cardinal step in order to determinate the correct diagnosis. We report the case of a man with a disseminated dermatosis affecting the right side of the body, characterized by brown papules linearly arranged in association to extracutaneous defects (leiomyomatosis peritonealis disseminata, bilateral brachydactylia, shortening of the right pelvic member, genu varum, hypoacusia). A histopathological study was performed showing findings compatible with the diagnosis of basaloid follicular hamartoma. Posteriorly he was diagnosed of symptomatic epilepsy. Basaloid follicular hamartoma is a rare follicular malformation that has been described as an isolated entity or in association with systemic alterations. Previous reports have described cases with similar characteristics to the one we are subjecting, and there is a proposition to group these cases as part of a spectrum of a distinct phenotype.