Non-advanced systemic mastocytosis (nonAdvSM) is poorly understood and likely often overlooked or misdiagnosed. Multiple issues complicate the accurate differential diagnosis of nonAdvSM subtypes. Recent updates for diagnostic criteria, as well as validated assessments and a greater understanding of laboratory findings for this population, are providing practitioners with additional tools for arriving at an accurate diagnosis. Although many therapeutics exist for addressing symptoms of nonAdvSM, those treatments are purely palliative. To date, no disease-modifying drugs have been FDA-approved for treating nonAdvSM. However, therapeutics that are already approved for advanced SM or other indications are being evaluated in the nonAdvSM space. These drugs, particularly KIT inhibitors, have the potential to improve patient outcomes for nonAdvSM. Healthcare providers are in a unique position to improve patient outcomes by becoming experts in diagnosing, assessing, and monitoring patients with nonAdvSM; as well as ensuring access to the latest, most effective treatments. To that end, there is a need to increase disease state awareness and close knowledge gaps around the diagnosis and management of this rare disease.