Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder marked by defects in lysosomal function that can manifest with a granulomatous enterocolitis resembling Crohn's disease (CD). We present a 16-year-old adolescent boy with HPS-associated CD and hidradenitis suppurativa (HS), representing one of a few cases of HPS with concurrent CD and HS described in the literature to date. Disease stabilization on combined infliximab and methotrexate highlights potentially shared inflammatory pathways involved in the pathogenesis of HPS-associated CD and HS. Given the rarity and refractory nature of this disease constellation, our case may provide a beneficial treatment strategy for other patients.